Rheumatology Last

Question 1
A 50-year-old female with a history of rheumatoid presents with a suspected septic knee joint. A
diagnostic aspiration is performed and sent to microbiology. Which of the following organisms is most
likely to be?
responsible?
A. Staphylococcus aureusia
B. Staphylococcus epidermidisia
C. Escherichia coliia
D. Neisseria gonorrhoeaeia
E. Streptococcus pneumoniaeia
Septic arthritis - most common organism: Staphylococcus aureus
Septic arthritis overview

Most common organism overall is Staphylococcus aureus in young adults who are sexually active
Neisseria gonorrhoeae should also be consider
Management
Synovial fluid should be obtained before starting treatment intravenous antibiotics which cover Gram-
positive cocci are indicated. The BNF currently recommends flucloxacillin + fusidic acid or clindamycin if
penicillin allergic antibiotic treatment is normally be given for several weeks (BNF states 6-12 weeks
(needle aspiration should be used to decompress the joint surgical drainage may be needed if frequent
needle aspiration is required
Question 2
Reactive arthritis is associated with which one of the following HLA antigens?
A. HLA-B27
B. HLA-A3
C. HLA-DR4
D. HLA-B5
E. HLA-DR3
Reactive arthritis
Reactive arthritis is one of the HLA-B27 associated seronegative spondylo arthropathies. It encompasses
Reiter's syndrome, a term which described a classic triad of urethritis, conjunctivitis and arthritis following
a dysenteric illness during the Second World War. Later studies identified patients who developed
symptoms following a sexually transmitted infection
)post-STI, now sometimes referred to as sexually acquired reactive arthritis,SARA(
The American College of Rheumatology now define reactive arthritis as an episode of peripheral arthritis
lasting for greater than 1 month associated with urethritis/cervicitis or diarrhoea
Epidemiology
Post-STI form much more common in men (e.g. 10:1( post-dysenteric form equal sex incidence
Organisms often responsible for post-dysenteric form
Shigella flexneri
Salmonella typhimurium
Salmonella enteritidis
Yersinia enterocolitica
Campylobacter
Organisms often responsible for post-STI form
Chlamydia trachomatis
Question 3
Low levels of which one of the following types of complement are associated with the development of
systemic lupus erythematous?
A.C4
B. C5
C. C6
D. C7
E. C8
SLE: C3 & C4 low

Low levels of C4a and C4b have been shown to be associated with an increased risk of developing
systemic lupus erythematous
SLE: investigations
Immunology
99% are ANA positive
20% are rheumatoid factor positive
anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
anti-Smith: most specific (> 99%), sensitivity (30%)
Monitoring
ESR: during active disease the CRP is characteristically normal - a
raised CRP may indicate underlying infection complement levels (C3, C4) are low during active disease
(formation of complexes leads to consumption of complement)anti-dsDNA titres can be used for disease
monitoring (but note not present in all patients)
Question 4
Which one of the following is least associated with Behcet's syndrome?
A. Mouth ulcers
B. Genital ulcers
C. Conjunctivitis
D. Deep vein thrombosis
E. Aseptic meningitis
Oral ulcers + genital ulcers + anterior uveitis = Behcet's

Mouth ulcers, genital ulcers, deep vein thrombosis and aseptic meningitis are all recognised features of
Behcet's syndrome
Ocular involvement is the most feared complication of Behcet's syndrome.
Conjunctivitis is seen rarely and is much less common than anterior uveitis.
Other ocular problems seen include retinal vasculitis, iridocyclitis and chorioretinitis
Behcet's syndrome
Behcet's syndrome is a complex multisystem disorder associated with presumed autoimmune mediated
inflammation of the arteries and veins. The precise aetiology has yet to be elucidated however. The
classic triads of symptoms are oral ulcers, genital ulcers and anterior uveitis
Epidemiology
More common in the eastern Mediterranean (e.g. Turkey) more common in men (complicated gender
distribution which varies according to country. Overall, Behcet's is considered to be more common and
more severe in men)
Tends to affect young adults (e.g. 20 - 40 years old)
Associated with HLA B5 and MICA6 allele
c.30% of patients have a positive family history
Features
Classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
Thrombophlebitis
Arthritis
Neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
Erythema nodosum, DVT, papulopostular rash
Diagnosis
No definitive test
Diagnosis based on clinical findings
Positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small
pustule forming)
Question 5
A 47-year-old man with a history of chronic sinusitis presents with shortness of breath to the Emergency
Department. Initial investigations reveal:
Hb 10.4g/dl
Platelets 477 * 109/l
WCC 14.3 * 109/l
ESR 92 mm/h
Urea 20 mmol/l
Creatinine 198 µmol/l
Urine dipstickblood +++
What is the most likely diagnosis?

A. Mixed cryoglobulinaemia
B. Churg-Strauss syndrome
C. Wegener's granulomatosis
D. Haemolytic uraemic syndrome
E.Henoch-Schonlein purpura
The combination of pulmonary and renal involvement combined with a history of chronic sinusitis points
towards a diagnosis of Wegener's granulomatosis
Wegener's granulomatosis is an autoimmune condition associated with a necrotizing granulomatous
vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys
Features
Upper respiratory tract: epistaxis, sinusitis, nasal crusting
Lower respiratory tract: dyspnoea, haemoptysis
Glomerulonephritis ('pauci-immune', 80% of patients)
Saddle-shape nose deformity
Also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions
Investigations
cANCA positive in > 90%, pANCA positive in 25%
Chest x-ray: wide variety of presentations, including cavitating lesions
Management
Steroids
Cyclophosphamide (90% response)
Plasma exchange
Median survival = 8-9 years
Question 6
A 34-year-old woman with a history of antiphospholipid syndrome presents with a swollen and painful leg.
Doppler ultrasound confirms a deep vein thrombosis (DVT). She had a previous DVT 4 months ago and
was taking warfarin (with a target INR of 2-3) when the DVT occurred. How should her anticoagulation be
managed?
A. Life-long warfarin, increase target INR to 3 - 4
B. Add in life-long low-dose aspirin
C. A further 6 months warfarin, target INR 2 - 3
D. A further 6 months warfarin, target INR 3 - 4
E. Life-long warfarin, target INR 2 - 3
The evidence base is scanty here but most clinicians would increase the target INR to 3-4 if a patient has
had a further thrombosis with an INR of 2-3. Please see the BCSH guidelines
Antiphospholipid syndrome is an acquired disorder characterised by a predisposition to both venous and
arterial thromboses, recurrent fetal loss and thrombocytopenia. It may occur as a primary disorder or
secondary to other conditions, most commonly systemic lupus erythematosus (SLE)
A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the
APTT. This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids
involved in the coagulation cascade
Features
Venous/arterial thrombosis
Recurrent fetal loss
Livedo reticularis
Thrombocytopenia
Prolonged APTT
Other features: pre-eclampsia, pulmonary hypertension
Associations other than SLE
Other autoimmune disorders
Lymphoproliferative disorders
Phenothiazines (rare)
Management - based on BCSH guidelines
Initial venous thromboembolic events: evidence currently supports use of warfarin with a target INR of 2-3
for 6 months.
Recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then
increase target INR to 3-4.
Arterial thrombosis should be treated with lifelong warfarin with target INR 2-3.
Question 7
Which of the following features are not typically seen in a patient with adult onset Still's disease?
A. Maculopapular rash
B. Rheumatoid factor
C. Pyrexiaia
D. High ferritin levelia
E. Lymphadenopathyia
Adult onset Still's disease is typically rheumatoid factor negative

Adult Still's disease typically affects 16-35 year olds
Features
Arthralgia
Elevated serum ferritin
Rash: salmon-pink, maculopapular, pruritic
Pyrexia
Lymphadenopathy
Rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative
Question 8
A 34-year-old intravenous drug user is admitted with a purpuric rash affecting her legs. Blood tests reveal
the following:
Hb 11.4g/dl
Platelets 489 * 109/l
WCC 12.3 * 109/l
HCV PCR positive
HBsAg negative
Rheumatoid factor positive
C3/C4 reduced

A. Polyarteritis nodosa
B. Henoch-Schonlein purpura
C. Wegener's granulomatosis
D. Cryoglobulinaemia
E. Systemic lupus erythematous
Hepatitis C infection is associated with type II (mixed) cryoglobulinaemia, suggested by

the purpuric rash, positive rheumatoid factor and reduced complement levels
Cryoglobulinaemia
Immunoglobulins which undergo reversible precipitation at 4 deg C,
dissolve when warmed to 37 deg C. One third of cases are idiopathic
Three types
Type I (25%): monoclonal
Type II (25%): mixed monoclonal and polyclonal: usually with RF
Type III (50%): polyclonal: usually with RF
Type I
Monoclonal - IgG or IgM
Associations: multiple myeloma, Waldenstrِm macroglobulinaemia
Type II
Mixed monoclonal and polyclonal: usually with RF
Associations: hepatitis C, RA, Sjogren's, lymphoma
Type III
Polyclonal: usually with RF
Associations: RA, Sjogren's
Symptoms (if present in high concentrations)
Raynaud's only seen in type I
Cutaneous: vascular purpura, distal ulceration, ulceration
Arthralgia
Renal involvement (diffuse glomerulonephritis)
Tests
Low complement (esp. C4)
High ESR
Treatment
Immunosuppression
Plasmapheresis
Question 9
Which one of the following is not a risk factor for developing osteoporosis?
A. Smoking
B. Obesity
C. Sedentary lifestyle
D. Premature menopause
E. Female sex
Low body mass, rather than obesity is associated with an increased risk of developing osteoporosis
Osteoporosis risk factors
Family history
Female sex
Increasing age
Deficient diet
Sedentary lifestyle
Smoking
Premature menopause
Low body weight
Asians and Orientals
Diseases which predispose
Endocrine: glucocorticoid excess (e.g. Cushing's, steroid therapy),hyperthyroidism, hypogonadism (e.g.
Turner's), growth hormone deficiency,hyperparathyroidism, diabetes mellitus.
Multiple myeloma, lymphoma
GI problems: malabsorption (e.g. coeliacs), gastrectomy, liver disease
Rheumatoid arthritis
Long term heparin therapy*
Chronic renal failure
Osteogenesis imperfecta, homocystinuria
*research is ongoing as to whether warfarin is a risk factor
Question 10
A 41-year-old man with a past history of asthma presents with pain and weakness in his left hand.
Examination findings are consistent with left ulnar nerve palsy. Blood tests reveal an eosinophilia. Which
one of the following antibodies is most likely to be present?
A. ANA
B. Anti-Scl70
C. pANCA
D. antiphospholipid antibodies
E. cANCA
This patient has Churg-Strauss syndrome as evidenced by the asthma, mononeuritis and eosinophilia
Churg-Strauss syndrome is an ANCA associated small-medium vessel vasculitis
Features
Asthma
Blood eosinophilia (e.g. > 10%)
Paranasal sinusitis
Mononeuritis multiplex
pANCA positive in 60%
Leukotriene receptor antagonists may precipitate the disease
Question 11
A health trust in the United Kingdom which serves a population of 100,000 is planning services for
patients with rheumatoid arthritis. How many of the population would be expected to have the disease?
A.100
B. 300
C. 1,000
D. 2,000
E. 10,000
The prevalence of rheumatoid arthritis in the UK population is approximately 1%
Rheumatoid arthritis: epidemiology
Peak onset = 30-50 years, although occurs in all age groups

F: M ratio = 3:1
Prevalence = 1%
Some ethnic differences e.g. high in Native Americans
Associated with HLA-DR4 (especially Felty's syndrome)
Question 12
If 59-year-old man with a history of gout presents with a swollen and painful first metatarsophalangeal
joint. He currently takes allopurinol 400mg od as gout prophylaxis. What should happen to his allopurinol
therapy?
A. Stop and recommence 4 weeks after acute inflammation has settled
B. Reduce allopurinol to 100mg od until acute attack has settled
C. Stop and switch to colchicine prophylaxis
D. Stop and recommence 2 weeks after acute inflammation has settled
E. Continue allopurinol in current dose
Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in
the synovium. It is caused by chronic hyperuricaemia (uric acid > 450 µmol/l)
Acute management
NSAIDs
Intra-articular steroid injection
Colchicine has a slower onset of action. The main side-effect is diarrhoea
If the patient is already taking allopurinol it should be continued
Allopurinol prophylaxis - see indications below
Allopurinol should not be started until 2 weeks after an acute attack has settled initial dose of 100 mg
od, with the dose titrated every few weeks to aim for a serum uric acid of < 300 µmol/l
NSAID or colchicine cover should be used when starting allopurinol month befor and after
Indications for allopurinol*
Recurrent attacks - the British Society for Rheumatology recommend 'In
Uncomplicated gout uric acid lowering drug therapy should be started if a
Second attack, or further attacks occur within 1 year'
Tophi
Renal disease
Uric acid renal stones
Prophylaxis if on cytotoxics or diuretics
*patients with Lesch-Nyhan syndrome often take allopurinol for life GHPRT enzyme defec.
Question 13
A 31-year-old female intolerant of methotrexate is started on azathioprine for rheumatoid arthritis. Routine
blood monitoring shows a Hb of 6.9, platelets of 72 and WBC of 2.5. Which of the following factors will
predispose her to azathioprine toxicity?
A. Cimetidine
B. Rifampicin
C. Fast acetylator status
D. Thiopurine methyltransferase deficiency
E. Alcohol excess
Thiopurine methyltransferase (TPMT) deficiency is present in c. 1 in 300 people and predisposes to

azathioprine related pancytopaenia
Azathioprine is metabolised to the active compound mercaptopurine, a purine analogue that inhibits
purine synthesis. A thiopurine methyltransferase (TPMT) test may be needed to look for individuals prone
to azathioprine toxicity
Adverse effects include
Bone marrow depression
Nausea/vomiting
Pancreatitis
A significant interaction may occur with allopurinol and lower doses of azathioprine should be used
Question 14
A 45-year-old female with a history of rheumatoid arthritis presents to the Emergency Department with a
two day history of a hot, painful, swollen right elbow joint. What is the most appropriate management?
A.Joint aspiration
B. Start infliximab
C. Oral high-dose prednisolone
D. Short course of methotrexate
E. Depomederone injection
Joint aspiration is mandatory in all patients with a hot, swollen joint to rule
out septic arthritis. If this was excluded in the above patient then intra-articular or system steroid therapy
may be considered.
Septic arthritis overview
Most common organism overall is Staphylococcus aureus in young adults who are sexually active
Neisseria gonorrhoeae should also be considered
Management
Synovial fluid should be obtained before starting treatment
Intravenous antibiotics which cover Gram-positive cocci are indicated. The BNF currently recommends
flucloxacillin + fusidic acid or clindamycin if penicillin allergic antibiotic treatment is normally be given
for several weeks (BNF states 6-12 weeks)
Needle aspiration should be used to decompress the joint
Surgical drainage may be needed if frequent needle aspiration is required
Question 15
Which one of the following statements regarding systemic lupus erythematous is true?
A.AIt is linked with HLA A5
B. Onset is typically between 20-40 years old
C. It is more common in Caucasians
D. The female: male ratio is 3:1
E. The incidence has decreased in the past 30 years
Systemic lupus erythematosus

Epidemiology
Much more common in females ( F:M = 9:1)
More common in Afro-Caribbeans* and Asian communities
Onset is usually 20-40 years
Incidence has risen substantially during the past 50 years (3 fold using
American College of Rheumatology criteria)
Pathophysiology
Autoimmune disease
Associated with HLA B8, DR2, DR3
Thought to be caused by immune system dysregulation leading to immune complex formation
Immune complex deposition can affect any organ; skin, joints, kidneys brain most commonly affected
*It is said the incidence in black Africans is much lower than in black Americans - the reasons for this are
unclear
Question 16
An autoantibody screen reveals that a patient is positive for anti-Jo 1 antibodies. What is the most likely
underlying diagnosis?
A. Limited cutaneous systemic sclerosis
B. Mixed connective tissue disease
C. Dermatomyositis
D. Polymyositis
E. Diffuse cutaneous systemic sclerosis
Anti-Jo 1 antibody are more commonly seen in polymyositis than dermatomyositis
Extractable nuclear antigens overview
Specific nuclear antigens usually associated with being ANA positive
Examples
Anti-Ro: Sjogren's syndrome, SLE, congenital heart block
Anti-La: Sjogren's syndrome
Anti-Jo 1: polymyositis
Anti-scl-70: diffuse cutaneous systemic sclerosis
Anti-centromere: limited cutaneous systemic sclerosis
Question 17
Which one of the following is least recognised as a risk factor for developing osteoporosis?
A. Cushing's syndrome
B. Turner's syndrome hypogonadism
C. Hyperparathyroidism
D. Hypothyroidism
E. Diabetes mellitus
Hyperthyroidism is associated with an increased risk of osteoporosis. Patients with hypothyroidism who
take excessive amounts of levothyroxine may also be at risk of osteoporosis
Family history
Female sex
Increasing age
Deficient diet
Sedentary lifestyle
Smoking
Premature menopause
Low body weight
Endocrine: glucocorticoid excess (e.g. Cushing's, steroid therapy),
Hyperthyroidism, hypogonadism (e.g. Turner's), growth hormone deficiency,
Hyperparathyroidism, diabetes mellitus
Question 18
Which one of the following features is least commonly seen in drug-induced lupus?
A. Glomerulonephritis
B. Arthralgia
C. Myalgia
D. Malar rash
E. Pleurisy
Glomerulonephritis is unusual in drug-induced lupus
Drug-induced lupus
In drug-induced lupus not all the typical features of systemic lupus erythematosus are seen, with renal
and nervous system involvement being unusual. It usually resolves on stopping the drug
Features
Arthralgia
Myalgia
Skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
ANA positive in 100%, dsDNA negative
Anti-Ro, anti-Smith positive in around 5%
Causes
Procainamide
Isoniazid
Minocycline
Hydralazine
Chlorpromazine
Anti-epileptics: phenytoin, anti TNF, methyldopa, D-penicillamine
Question 19
A 79-year-old man presents with a history of lower back pain and right hip pain. Blood tests reveal the
following:
Calcium 2.20 mmol/l
Phosphate 0.8 mmol/l
ALP 890 u/L
A. Primary hyperparathyroidism
B. Chronic renal failure
C. Osteomalacia
D. Osteoporosis
E. Paget's disease
The normal calcium and phosphate combined with a raised alkaline phosphate points to a diagnosis of
Paget's
Paget's disease is a disease of increased but uncontrolled bone turnover. It is thought to be primarily a
disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic
activity. Paget's disease is common (UK prevalence 5%) but symptomatic in only 1 in 20 patients
Predisposing factors
Increasing age
Male sex
Northern latitude
Family history
Clinical features - only 5% of patients are symptomatic
Bone pain (e.g. pelvis, lumbar spine, femur)
Classical, untreated features: bowing of tibia, bossing of skull
Raised alkaline phosphatase (ALP) - calcium* and phosphate are typically normal
Skull x-ray: thickened vault, osteoporosis circumscripta
Indications for treatment include bone pain, skull or long bone deformity, fracture, periarticular Paget's
Bisphosphonate (either oral risedronate or IV zoledronate) alendronate oral
Calcitonin is less commonly used now
Complications
Deafness (cranial nerve entrapment)
Bone sarcoma (1% if affected for > 10 years)
Fractures
Skull thickening
High-output cardiac failure
*usually normal in this condition but hypercalcaemia may occur with prolonged immobilisation
Question 20
A 62-year-old female presents with a two week history of intermittent headaches and lethargy. Blood tests
reveal the following:
ESR 67 mm/hr
A. Polymyalgia rheumatica
B. Cluster headaches
C. Polyarteritis nodosa
D. Migraine
E. Temporal arteritis
This is a classic history of temporal arteritis. Treatment should be started immediately with high dose
steroids (e.g. prednisolone 1mg/kg/day) to reduce the chance of visual loss
Temporal arteritis is large vessel vasculitis which overlaps with polymyalgia rheumatica (PMR).
Histology shows changes which characteristically 'skips' certain sections of affected artery whilst
damaging others
Features
Typically patient > 60 years old
Usually rapid onset (e.g. < 1 month)
Headache (found in 85%)
Jaw claudication (65%)
Tender, palpable temporal artery
Features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
Also lethargy, depression, low-grade fever, anorexia, night sweats
Investigations
ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
Temporal artery biopsy: skip lesions may be present
Note CK and EMG normal
Reduced CD8+ T cells
Treatment High-dose prednisolone (dramatic response)
Question 21
A 57-year-old man presents with pain in his right knee. An x-ray shows osteoarthritis. He has no past
medical history of note. What is the most suitable treatment option for the management of his pain?
A. Diclofenac with omeprazole
B. Glucosamine
C. Diclofenac
D. Ibuprofen
E. Paracetamol
Osteoarthritis - paracetamol + topical NSAIDs (if knee/hand) first-line

Oral NSAIDs should be used second line in osteoarthritis due to their adverse effect profile
NICE published guidelines on the management of osteoarthritis (OA) in 2008
- All patients should be offered help with weight loss, given advice about local muscle strengthening
exercises and general aerobic fitness
- Paracetamol and topical NSAIDs are first-line analgesics. Topical NSAIDs are indicated only for OA of
the knee or hand
- Second-line treatment is oral NSAIDs/COX-2 inhibitors, opioids, capsaicin cream and intra-articular
corticosteroids. A proton pump inhibitor should be co-prescribed with either drug. These drugs should be
avoided if the patient takes aspirin
- Non-pharmacological treatment options include supports and braces, TENS and shock absorbing insoles
or shoes
- If conservative methods fail then refer for consideration of joint replacement
What is the role of glucosamine?
Normal constituent of glycosaminoglycans in cartilage and synovial fluid a systematic review of several
double blind RCTs of glucosamine in knee osteoarthritis reported significant short-term symptomatic
benefits including significantly reduced joint space narrowing and improved pain scores more recent
studies have however been disappointing the 2008 NICE guidelines suggest it is not recommended
Question 22
Perinuclear antineutrophil cytoplasmic antibodies (pANCA) are most stronglyassociated with which
condition?
A. Goodpasture's syndrome
B. Churg-Strauss syndrome
C. Polyarteritis nodosa
D. Wegener's granulomatosis
E. Autoimmune hepatitis
cANCA = Wegener's; pANCA = Churg-Strauss + others
ANCA
There are two main types of anti-neutrophil cytoplasmic antibodies (ANCA) - cytoplasmic (cANCA) and
perinuclear (pANCA)
For the exam, remember:
cANCA - Wegener's granulomatosis
pANCA - Churg-Strauss syndrome + others (see below)
cANCA
Most common target serine proteinase 3 (PR3)
Some correlation between cANCA levels and disease activity
Wegener's granulomatosis, positive in > 90%
Microscopic polyangiitis, positive in 40%
pANCA
Most common target is myeloperoxidase (MPO)
Cannot use level of pANCA to monitor disease activity
Associated with immune crescentic glomerulonephritis (positive in c. 80% of patients)
Microscopic polyangiitis, positive in 50-75%
Churg-Strauss syndrome, positive in 60%

Wegener's granulomatosis, positive in 25%
Other causes of positive ANCA (usually pANCA)
Inflammatory bowel disease (UC > Crohn's)
Connective tissue disorders: RA, SLE, Sjogren's
Autoimmune hepatitis
Question 23
Which of the following is associated with a good prognosis in rheumatoid arthritis?
A. Rheumatoid factor negative
B. HLA DR4
C. Female sex
D. Rheumatoid nodules
E. Insidious onset
Rheumatoid arthritis poor prognostic features:-

Poor functional status at presentation
HLA DR4
X-ray: early erosions (e.g. after < 2 years)
Extra articular features e.g. nodules
Female sex
Insidious onset
Anti-CCP antibodies
Question 24
A 25-year-old woman presents with a three day history of dysuria and a painful left knee. During the
review of symptoms she mentions a bout of diarrhoea and crampy abdominal pain three weeks ago. She is
normally fit and well and takes no regular medication. Her father died of colorectal cancer in his sixth
decade. On examination the left knee is red, swollen and hot to touch. What is the most likely diagnosis?
A. Reactive arthritis secondary to Salmonella spp.
B. Reactive arthritis secondary to Chlamydia trachomatis
C. Rheumatoid arthritis
D. Ulcerative colitis
E. Gonococcal arthritis
Urethritis + arthritis + conjunctivitis = reactive arthritis

Two of the classic three features of reactive arthritis (urethritis, arthritis and conjunctivitis) are present in
this patient. The family history of colorectal cancer is of no particular significance
Reactive arthritis is one of the HLA-B27 associated seronegative spondyloarthropathies. It encompasses
Reiter's syndrome, a term which described a classic triad of urethritis, conjunctivitis and arthritis following
a dysenteric illness during the Second World War. Later studies identified patients who developed
symptoms following a sexually transmitted infection (post-STI, now sometimes referred to as sexually
acquired reactive arthritis, SARA)
The American College of Rheumatology now define reactive arthritis as an episode of peripheral
arthritis lasting for greater than 1 month associated with urethritis/cervicitis or diarrhoea
Epidemiology
Post-STI form much more common in men (e.g. 10:1)
Post-dysenteric form equal sex incidence
Organisms often responsible for post-dysenteric form
Shigella flexneri
Salmonella typhimurium
Salmonella enteritidis
Yersinia enterocolitica
Campylobacter
Organisms often responsible for post-STI form
Chlamydia trachomatis
Question 25
Which one of the following cytokines is the most important in thepathophysiology of rheumatoid arthritis?
A. IFN-beta
B. IFN-alpha
C. IL-4
D. Tumour necrosis factor
E. IL-2
Rheumatoid arthritis - TNF is key in pathophysiology

Tumour necrosis factor (TNF) is a pro-inflammatory cytokine with multiple roles in the immune system
- TNF is secreted mainly by macrophages and has a number of effects on the immune system, acting
mainly in a paracrine fashion:
*Activates macrophages and neutrophils
*Acts as costimulator for T cell activation
*Key mediator of bodies response to Gram negative septicaemia
*Similar properties to IL-1
*Anti-tumour effect (e.g. phospholipase activation)
*TNF-alpha binds to both the p55 and p75 receptor. These receptors can induce apoptosis. It also cause
activation of NFkB
*Endothelial effects include increase expression of selectins and increased production of platelet activating
factor, IL-1 and prostaglandins
*TNF promotes the proliferation of fibroblasts and their production of protease and collagenase. It is
thought fragments of receptors act as binding points in serum
*Systemic effects include pyrexia, increased acute phase proteins and disordered metabolism leading to
cachexia
*TNF is important in the pathogenesis of rheumatoid arthritis - TNF blockers (e.g. infliximab, etanercept)
are now licensed for treatment of severe rheumatoid
TNF blockers
Infliximab: monoclonal antibody, IV administration
Infliximab is also used in active Crohn's disease unresponsive to steroids
Etanercept: fusion protein that reversibly binds soluble TNF receptors, subcutaneous administration
Adalumimab: monoclonal antibody, subcutaneous administration
Adverse effects of TNF blockers include reactivation of latent tuberculosis and

demyelination
Question 26
A 47-year-old female presents with elbow pain. She has just spent the weekend painting the house. On
examination there is localised pain around the lateral epicondyle and a diagnosis of lateral epicondylitis is
suspected. Which one of the following movements would characteristically worsen the pain?
A. Resisted thumb flexion
B. Thumb extension
C. Flexion of the elbow
D. Pronation of the forearm with the elbow flexed
E. Resisted wrist extension with the elbow extended
Lateral epicondylitis: worse on resisted wrist extension/suppination whilst elbow extended

Lateral epicondylitis typically follows unaccustomed activity such as housepainting or playing tennis
('tennis elbow')
Features
Pain localised to the lateral epicondyle
Pain worse on resisted wrist extension with the elbow extended or supination of the forearm with the
elbow extended
Question 27
A 54-year-old female is reviewed in the rheumatology clinic due to dry eyes and arthralgia. A diagnosis of
primary Sjogren's syndrome is suspected. Which one of the following features is least associated with this
condition?
A. Renal tubular acidosis
B. Xerostomia
C. Sensory polyneuropathy
D. Dilated cardiomyopathy
E. Raynaud's phenomenon
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal
surfaces. It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders,
where it usually develops around 10 years after the initial onset. Sjogren's syndrome is much more
common in females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold)
Features
Dry eyes: keratoconjunctivitis sicca
Dry mouth
Vaginal dryness
Arthralgia
Raynaud's, myalgia
Sensory polyneuropathy
Renal tubular acidosis (usually subclinical)
Investigation
Rheumatoid factor (RF) positive in more than 80%
ANA positive in 70%
Anti-Ro (SSA) antibodies in 70% of patients with PSS
Anti-La (SSB) antibodies in 30% of patients with PSS
Schirmer's test: filter paper near conjunctival sac to measure tears formation <10mm in 5 min. +ve test
Histology: focal lymphocytic infiltration
Also: hypergammaglobulinaemia, low C4
Management
Artificial saliva and tears
Pilocarpine may stimulate saliva production
Question 28
Which one of the following is the most common ocular manifestation of rheumatoid arthritis?
A. Scleritis
B. Episcleritis
C. Keratoconjunctivitis sicca
D. Corneal ulceration
E. Keratitis
Keratoconjunctivitis sicca is characterised by dry, burning and gritty eyes caused by decreased tear
production
Rheumatoid arthritis: Ocular manifestations of rheumatoid arthritis are common, with 25% of patients
having eye problems
Ocular manifestations
Keratoconjunctivitis sicca (most common)
Episcleritis (erythema)
Scleritis (erythema and pain)
Corneal ulceration
Keratitis
Iatrogenic
Chloroquine retinopathy
Steroid-induced cataracts
Question 29
Which of the following findings is not typical in a patient with antiphospholipid syndrome?
A. Prolonged APTT
B. Thrombocytosis
C. Recurrent venous thrombosis
D. Recurrent arterial thrombosis
E. Livedo reticularis
Antiphospholipid syndrome: arterial/venous thrombosis, miscarriage, livedo reticularis
Thrombocytopenia is associated with antiphospholipid syndrome.
Features
Livedo reticularis
Thrombocytopenia
Prolonged APTT
Initial venous thromboembolic events: evidence currently supports use of warfarin with a target INR of 2-3
for 6 months.
Recurrent venous thromboembolic events: lifelong warfarin;
if occurred whilst taking warfarin then increase target INR to 3-4.
Arterial thrombosis should be treated with lifelong warfarin with target INR 2-3.
Question 30
Osteopetrosis is due to a defect in:
A. Osteoclast function
B. PTH receptors
C. Osteoblast function
D. Calcium resorption in proximal tubule
E. Calcium absorption
Osteopetrosis overview
Aka marble bone disease
Rare disorder of defective osteoclast function resulting in failure of normal bone resorption
Stem cell transplant and interferon-gamma have been used for treatment
Question 31
Which one of the following is not part of the American College of Rheumatology criteria for diagnosing
rheumatoid arthritis?
A. Raised ESR or CRP
B. Morning stiffness > 1 hr
C. Subcutaneous nodules
D. Symmetrical arthritis
E. Rheumatoid factor positive
Rheumatoid arthritis: ACR criteria (American College of Rheumatology criteria)
Requires 4 of the following 7 criteria Sensitivity = 92%, specificity = 89%
- Morning stiffness > 1 hr (for at least 6 weeks)
- Soft-tissue swelling of 3 or more joints (for at least 6 weeks)
- Swelling of PIP, MCP or wrist joints (for at least 6 weeks)
- Symmetrical arthritis
- Subcutaneous nodules
- Rheumatoid factor positive
- Radiographic evidence of erosions or periarticular osteopenia
3SSS (6 wk.), R2X

Question 32
A 64-year-old man presents to the Emergency Department with a 2 day history of a swollen, painful left
knee. Aspirated joint fluid shows calcium pyrophosphate crystals. Which of the following blood tests is
most useful in revealing an underlying cause?
A. Transferrin saturation
B. ACTH
C. ANA
D. Serum ferritin
E. LDH
This is a typical presentation of pseudogout. Elevated transferrin saturation may indicate
haemochromatosis, a recognised cause of pseudogout.
A high ferritin level is also seen in haemochromatosis but can be raised in a variety of infective and
inflammatory processes, including pseudogout, as part of an acute phase response
Pseudogout
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate
dihydrate in the synovium
Features
Knee, wrist and shoulders most commonly affected
X-ray: chondrocalcinosis
Risk factors
Hyperparathyroidism
Hypothyroidism
Haemochromatosis
Acromegaly
Low magnesium, low phosphate
Wilson's disease
Management
Aspiration of joint fluid, to exclude septic arthritis and show weakly-positively birefringent brick shaped
crystals.
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
Question 33
Which one of the following antibodies is most specific for limited cutaneous systemic sclerosis?
A. Anti-Jo 1antiobodies
B. Rheumatoid factor
C. Anti-Scl-70 antibodies
D. Anti-centromere antibodies
E. Anti-nuclear factor
Limited (central) systemic sclerosis = anti-centromere antibodies
Although ANA is positive in 90% of patients with systemic sclerosis, anti-centromere antibodies are the
most specific test for limited cutaneous systemic sclerosis
Systemic sclerosis
Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other
connective tissues. It is four times more common in females
There are three patterns of disease:
Limited cutaneous systemic sclerosis
Raynaud's may be first sign
Scleroderma affects face and distal limbs predominately
Associated with anti-centromere antibodies
A subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon,
oEsophageal dysmotility, Sclerodactyly, Telangiectasia
Diffuse cutaneous systemic sclerosis

Scleroderma affects trunk and proximal limbs predominately
Associated with scl-70 antibodies topoismerase
Hypertension, lung fibrosis and renal involvement seen
Poor prognosis
Scleroderma (without internal organ involvement)
Tightening and fibrosis of skin may be manifest as plaques (morphoea) or linear
Antibodies
ANA positive in 90%
RF positive in 30%
Anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
Anti-centromere antibodies associated with limited cutaneous systemic
Sclerosis
Question 34
A 45-year-old woman is referred to rheumatology outpatients with a 4 month history of joint pains,
myalgia and generalised lethargy. An autoantibody screen reveals she is ANA positive and ribonuclear
protein positive. The creatine kinase is elevated at 525. What is the most likely diagnosis?
A. Systemic lupus erythematous
B. Mixed connective tissue disease
C. Polymyositis
D. Dermatomyositis
E. CREST syndrome
Anti-ribonuclear protein (anti-RNP) = mixed connective tissue
disease
Mixed connective tissue disease

Features of SLE, systemic sclerosis and polymyositis
Anti-RNP positive
Question 35
Which of the following is least likely to be associated with ankylosing spondylitis?
A. Apical fibrosis
B. Achilles tendonitis
C. Amyloidosis
D. Achalasia
E. Heart block
Ankylosing spondylitis features - the 'A's
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
Achalasia is not a recognised association of ankylosing spondylitis
Ankylosing spondylitis:
Features
Typically a young man who presents with lower back pain and stiffness
Stiffness is usually worse in morning and improves with activity
Peripheral arthritis (25%, more common if female)
Other features - the 'A's
Apical fibrosis
Anterior uveitis
Achilles tendonitis
AV node block
Amyloidosis
And cauda equina syndrome
X-rays
Are often normal early in disease, later changes include:
Sacroilitis: subchondral erosions, sclerosis
Squaring of lumbar vertebrae
'Bamboo spine' (late & uncommon) calcification of interspinous ligaments,, and sendesmophyte
Chest x-ray: apical fibrosis
Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and
ankylosis of the costovertebral joints
Question 36
A 54-year-old man with a history of type 2 diabetes mellitus presents with a history of right shoulder pain.
On examination there is limited movement of the right shoulder in all directions. What is the most likely
diagnosis?
A. Adhesive capsulitis
B. Dermatomyositis
C. Avascular necrosis
D. Lhermitte's syndrome
E. Diabetic amyotrophy
Diabetic amyotrophy affects the lower limbs

Adhesive capsulitis
Adhesive capsulitis has a known association with diabetes. Patients typically have a painful
freezing phase, an adhesive phase and a recovery phase. The episode typically lasts 2-3 years.
Question 37
A 51-year-old male presents with an acute onset of swelling and pain in his right knee. Aspiration shows
negatively birefringent crystals with no organisms seen. His pain fails to settle with NSAIDs. What is the
most appropriate next step in his management?
A. Repeat joint aspiration and intra-articular depomederone methylprednisolone
B. Allopurinol
C. IV flucloxacillin
D. Diuretics
E. Low dose methotrexate
Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the
synovium. It is caused by chronic hyperuricaemia (uric acid > 450 µmol/l)
Acute management
NSAIDs
Colchicine has a slower onset of action. The main side-effect
is diarrhoea
If the patient is already taking allopurinol it should be
continued
Allopurinol prophylaxis - see indications below
Allopurinol should not be started until 2 weeks after an acute attack has settled initial dose of 100 mg
od, with the dose titrated every few weeks to aim for a serum uric acid of < 300 µmol/l
NSAID or colchicine cover should be used when starting allopurinol 4wk. befor and after
Recurrent attacks - the British Society for Rheumatology recommend 'In uncomplicated gout uric acid
lowering drug therapy should be started if a second attack, or further attacks occur within 1 year'
Tophi
Renal disease
*patients with Lesch-Nyhan syndrome often take allopurinol for life
Question 38
A 57-year-old man with a history of ischaemic heart disease presents with a hot, erythematous and painful
left metatarsophalangeal joint. The attack settles following a course of non-steroidal anti-inflammatories.
He currently takes aspirin 75 mg od for secondary prevention of ischaemic heart disease. What should
happen regarding his medication?
A. Switch aspirin to clopidogrel
B. Continue aspirin at current dose
C. Increase aspirin dose to 300mg od
D. Switch aspirin to dipyridamole
E. Stop aspirin
Aspirin in a dose of 75-150mg is not thought to have a significant effect on plasma urate levels - see below
synovium. It is caused by chronic hyperuricaemia (uric acid > 0.45 mmol/l)
Drug causes
Thiazides, frusemide diuritics
Alcohol
Cytotoxic agents cytotoxic
Pyrazinamide
Question 39
A 31-year-old female with a history of SLE gives birth following a 39 week pregnancy. The newborn is
noted to be bradycardic. Which one of the following autoantibodies is associated with congenital heart
block?
A. Anti-Ro
B. Anti-scl-70
C. Anti-RNP
D. AMA
E. Anti-Jo 1
SLE: pregnancy overview

Risk of maternal autoantibodies crossing placenta leads to condition termed neonatal lupus erythematous
Neonatal complications include congenital heart block
Strongly associated with anti-Ro (SSA) antibodies
Question 40
Which one of the following is least recognised in polyarteritis nodosa?
A. Cytoplasmic-antineutrophil cytoplasmic antibodies
B. Hypertension
C. Mononeuritis multiplex
D. Pyrexia
E. Renal failure
Perinuclear-antineutrophil cytoplasmic antibodies are found in around 20% of patients

Polyarteritis nodosa
Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation
leading to aneurysm formation. PAN is more common in middle-aged men and is associated with
hepatitis B infection
Features
Fever, malaise, arthralgia
Hypertension
Mononeuritis multiplex, sensorimotor polyneuropathy
Haematuria, renal failure
Testicular pain
Abdominal pain (e.g. from mesenteric ischaemia)
Perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with
'classic' PAN
Question 41
A 61-year-old female is noted to have multiple sites of muscle tenderness in the rheumatology clinic. A
diagnosis of fibromyalgia is suspected. What is the most appropriate management?
A. Naproxen
B. Amitriptyline
C. Cognitive behavioural therapy
D. Propranolol
E. Prednisolone
Management of fibromyalgia may consist of the following:

Explanation
Increase in aerobic exercise
Low-dose amitriptyline
Question 42
A 28-year-old man is investigated for recurrent lower back pain. A diagnosis of ankylosing spondylitis is
suspected. Which one of the following investigations is most useful?
A. ESR
B. X-ray of the sacro-iliac joints
C. HLA-B27 testing
D. X-ray of the thoracic spine
E. CT of the lumber spine
X-ray of the sacro-iliac joints is the most useful investigation for diagnosis and monitoring, but changes
may not be seen for many years after the onset of symptoms
Ankylosing spondylitis
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathies. It typically presents in males (sex
ratio 5:1) aged 20-30 years old
HLA-B27 is of little use in making the diagnosis as it is positive in:

90% of patients with ankylosing spondylitis
10% of normal patients
Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis
Management (NICE in 2008)

NSAIDs
Physiotherapy
Sulphasalazine may be useful if there is peripheral joint involvement - doesn't improve spinal mobility
TNF-alpha blockers such as etanercept and adalimumab are increasingly used.
Question 43
A 54-year-old male presents with weakness of his upper arms. On examination he is found to have a
macular rash over his back and the extensor aspects of his upper arms. He is a heavy smoker and his
sodium is 121 mmol/l. What is the most likely underlying diagnosis?
A. Addison's disease
B. Polymyositis
C. Overlap syndrome
D. Dermatomyositis
E. Hypothyroidism
This man may have an underlying small cell lung cancer causing SIADH
Dermatomyositis overview
Inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions may
be idiopathic or associated with connective tissue disorders or underlying malignancy (found in 20-25% -
more if patient older)
Polymyositis is a variant of the disease where skin manifestations are not prominent
Skin features
Photosensitive
Macular rash over back and shoulder
Heliotrope rash over cheek
Gottron's papules - roughened red papules over extensor surfaces of fingers
Nail fold capillary dilatation
Other features
Proximal muscle weakness +/- tenderness
Raynaud's
Respiratory muscle weakness
Interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
Dysphagia, dysphonia
Question 44
Which one of the following conditions is least associated with HLA-B27?
A. Reiter's syndrome
B. Psoriatic arthritis
C. Ankylosing spondylitis
D. Crohn's disease
E. Sacroilitis
There is an indirect association between HLA-B27 and Crohn's as some patients may develop enteropathic
arthritis, but this is the least common association of the above
Seronegative spondyloarthropathies common features
Associated with HLA-B27
Rheumatoid factor negative - hence 'seronegative'
Peripheral arthritis, usually asymmetrical
Sacroilitis
Enthesopathy: e.g. Achilles tendonitis, plantar fasciitis
Extra-articular manifestations: uveitis, pulmonary fibrosis (upper zone),
Amyloidosis, aortic regurgitation
Spondyloarthropathies
Psoriatic arthritis
Reiter's syndrome (including reactive arthritis)
Enteropathic arthritis (associated with IBD)
Question 45
Which one of the following statements concerning discoid lupus is correct?
A. Commonly progresses to SLE
B. Causes non-scarring alopecia
C. Characterised by follicular keratin plugs
D. Is rarely photosensitive
E. Typically presents in older males
Discoid lupus erythematosus is characterised by follicular keratin plugs
Discoid lupus erythematous is a benign disorder generally seen in younger

females. It very rarely progresses to systemic lupus erythematosus (in less than 5% of cases).
Discoid lupus erythematous is characterised by follicular keratin plugs and is thought to be autoimmune in
aetiology
Features
Erythematous, raised rash, sometimes scaly
May be photosensitive
More common on face, neck, ears and scalp
Lesions heal with atrophy, scarring (may cause scarring alopecia), and pigmentation
Management
Topical steroid cream
Oral antimalarials may be used second-line e.g. hydroxychloroquine
Avoid sun exposure
Question 46
A 45-year-old woman with a history of primary Sjogren's syndrome is reviewed in clinic. Her main
problem is a dry mouth, which unfortunately has not responded to artificial saliva. Which one of the
following medications is most likely to be beneficial?
A. Rivastigmine
B. Neostigmine
C. Clonidine
D. Atropine
E. Pilocarpine
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.
It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it
usually develops around 10 years after the initial onset. Sjogren's syndrome is much more common in
females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold)
Features
Dry mouth
Vaginal dryness
Arthralgia
Raynaud's, myalgia
Investigation
ANA positive in 70%
Schirmer's test: filter paper near conjunctival sac to measure tears formation
Management
Question 47
Which one of the following is least recognised as a risk factor for developing osteoporosis?
A. Multiple myeloma
B. Rheumatoid arthritis
C. Long-term phenytoin therapy
D. Chronic renal failure
E. Diabetes mellitus
Long-term phenytoin therapy may cause enhanced vitamin D metabolism leading to osteomalacia, rather
than osteoporosis
Osteoporosis: Risk factors

Family history
Female sex
Increasing age
Deficient diet
Sedentary lifestyle
Smoking
Premature menopause
Low body weight
Hyperthyroidism, hypogonadism (e.g. Turner's), growth hormone deficiency,
Hyperparathyroidism, diabetes mellitus
Question 48
Which one of the following drugs is least likely to cause gout?
A. Lithium
B. Bendrofluazide
C. Alcohol
D. Pyrazinamide
E. Frusemide
Lithium was actually used to treat gout in the 19th century

Drug causes
Thiazides, frusemide
Alcohol
Cytotoxic agents
Pyrazinamide
Question 49
Which of the following is not a recognised cause of Raynaud's phenomenon?
A. Oral contraceptive pill
B. Cervical rib
C. Type I cryoglobulinaemia
D. Pizotifen
E. Scleroderma
Ergotamine rather than pizotifen is associated with Raynaud's phenomenon

Raynaud's phenomena may be primary (Raynaud's disease) or secondary (Raynaud's phenomenon)
Raynaud's disease typically presents in young women (e.g. 30 years old) with symmetrical attacks
Factors suggesting underlying connective tissue disease
Onset after 40 years
Unilateral symptoms
Rashes
Presence of autoantibodies
Digital ulcers, calcinosis
Very rarely: chilblains
Secondary causes
Connective tissue disorders: scleroderma (most common), rheumatoid arthritis, SLE
Leukaemia
Type I cryoglobulinaemia, cold agglutinins
Use of vibrating tools
Drugs: oral contraceptive pill, ergot

Cervical rib
Management
Calcium channel blockers
IV prostacyclin infusions
As pul. HTN treatment
Question 50
Which one of the following is true regarding the investigation of a patient with dermatomyositis?
A. Creatine kinase is characteristically normal
B. Muscle biopsy is contraindicated
C. Anti-Jo-1 antibodies are usually negative
D. Antinuclear antibodies are always negative
E. EMG is normal
Anti-Jo-1 antibodies are more

common in polymyositis than dermatomyositis
Dermatomyositis: Investigations
Elevated creatine kinase
EMG
Muscle biopsy
Anti-Jo-1 antibodies are not commonly seen in dermatomyositis - they are more common in polymyositis
where they are seen in a pattern of disease associated with lung involvement,
Raynaud's and fever
ANA positive in 60%
Management
Prednisolone
Question 51
Each one of the following is seen in reactive arthritis, except:
A. Urethritis
B. Keratoderma blenorrhagica
C. Conjunctivitis
D. Aseptic meningoencephalitis
E. Circinate balanitis
Reactive arthritis is one of the HLA-B27 associated seronegative spondyloarthropathies. Hans Reiter
described a classic triad is of urethritis, conjunctivitis and arthritis following a dysenteric illness during the
Second World War. Later studies identified patients who developed symptoms following a sexually
transmitted infection. The American College of Rheumatology now define reactive arthritis as an episode
of peripheral arthritis lasting for greater than 1 month associated with urethritis/cervicitis or diarrhoea
Features
Typically develops within 4 weeks of initial infection - symptoms generally last around 4-6 months
Arthritis is typically an asymmetrical oligoarthritis of lower limbs may present as monoarthritis e.g. knee
Symptoms of urethritis
Eye: conjunctivitis (seen in 50%), anterior uveitis
Skin: circinate balanitis (painless vesicles on the coronal margin of the prepuce),
keratoderma blenorrhagica (waxy yellow papules on palms and soles)
Around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease
Question 52
A 50 -year-old man with no past medical history is investigated for ongoing back pain. He is found to
have a vertebral collapse secondary to osteoporosis. What is the most appropriate test to determine the
cause of his osteoporosis?
A. Thyroid function tests
B. Prostate specific antigen
C. Oestrogen level
D. Prolactin level
E. Testosterone level
Whilst thyrotoxicosis is a known cause of osteoporosis, testosterone deficiency is much more likely in a
middle-aged male
Family history
Female sex
Increasing age
Deficient diet
Sedentary lifestyle
Smoking
Premature menopause
Low body weight
Hyperthyroidism, hypogonadism (e.g. Turner's), growth hormone deficiency, hyperparathyroidism,
diabetes mellitus
Question 53
Which one of the following is the most common type of psoriatic arthritis?
A. Monoarthritis
B. Sacroilitis
C. DIP joint disease
D. Rheumatoid-like polyarthritis
E. Arthritis mutilans
Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the development of
skin lesions. Around 10% percent of patients with skin lesions develop an arthropathy with males and
females being equally affected
Types*
Rheumatoid-like polyarthritis: (30-40%, most common type)
Asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
Sacroilitis
DIP joint disease (10%)
Arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')
Management
Treat as rheumatoid arthritis but better prognosis
*Until recently it was thought asymmetrical oligoarthritis was the most common type, based on data from
the original 1973 Moll and Wright paper.
Question 54
A 44-year-old female with a history of Raynaud's phenomenon is reviewed in the rheumatology clinic. She
is currently being investigated for dysphagia. On examination she is noted to have tight, shiny skin over
her fingers. Which one of the following complications is she most likely to develop?
A. Early onset dementia
B. Erythema nodosum
C. Malabsorption bacterial over growth
D. Constrictive pericarditis
E. Erosive joint disease
This patient is likely to have CREST syndrome, a subtype of limited cutaneous systemic sclerosis.
Malabsorption can develop in these patients secondary to bacterial overgrowth of the sclerosed small
intestine
Whilst diffuse systemic sclerosis is associated with more severe and rapid internal organ involvement it is
also seen in the limited form.
Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other
connective tissues. It is four times more common in females
There are three patterns of disease:
Limited cutaneous systemic sclerosis
Raynaud's may be first sign
Scleroderma affects face and distal limbs predominately
Associated with anti-centromere antibodies
A subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon,
oEsophageal dysmotility, Sclerodactyly, Telangiectasia
Diffuse cutaneous systemic sclerosis
Scleroderma affects trunk and proximal limbs predominately
Associated with scl-70 antibodies
Hypertension, lung fibrosis and renal involvement seen
Poor prognosis
Scleroderma (without internal organ involvement)
Tightening and fibrosis of skin
May be manifest as plaques (morphoea) or linear
Antibodies
ANA positive in 90%
RF positive in 30%
Anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
Anti-centromere antibodies associated with limited cutaneous systemic sclerosis
Question 55
Which one of the following is most useful in the management of Familial Mediterranean Fever?
A. Prednisolone
B. Erythromycin
C. Cyclophosphamide
D. Colchicine
E. Benzylpenicillin
Familial Mediterranean fever (FMF, also known as recurrent polyserositis) is an autosomal recessive
disorder which typically presents by the second decade. It is more common in people of Turkish, Armenian
and Arabic descent
Features - attacks typically last 1-3 days
Pyrexia
Abdominal pain (due to peritonitis)
Pleurisy
Pericarditis
Arthritis
Erysipeloid rash on lower limbs
Management
Colchicine may help
Question 56
Which one of the following is not associated with carpal tunnel syndrome?
A. Tinel's sign
B. Compression of the median nerve
C. Wasting of the hypothenar eminence
D. Flexion of the wrist reproduces symptoms
E. Weakness of thumb abduction
Carpal tunnel syndrome is caused by compression of median nerve in the carpal tunnel
History
Pain/pins and needles in thumb, index, middle finger e.g. at night
Patient flicks hand to obtain relief
Examination
Weakness of thumb abduction
Weakness touching base of little finger
Wasting of thenar eminence (NOT hypothenar)
Tinel's sign: tapping causes paraesthesia
Phalen's sign: flexion of wrist causes symptoms
Causes: - pregnancy, premenstrual, oedema (CCF), lunate fracture, RA
Electrophysiology: - motor + sensory: prolongation of the action potential
Treatment: diuretics, carpal injection, wrist splints at night, decompression (flexor retinaculum division)
Question 57
A 24-year-old female is investigated for intermittent pain and swelling of the metacarpal phalangeal joints
for the past 3 months. An x-ray shows loss of joint space and soft-tissue swelling. Rheumatoid factor is
positive and a diagnosis of rheumatoid arthritis is suspected. What is the most appropriate management?
A. Infliximab
B. Azathioprine
C. Sulfasalazine
D. Methotrexate
E. Diclofenac
In the future anti-TNF drugs may become first-line but they are currently used when standard disease
modifying drugs fail to control the disease process
Rheumatoid arthritis: management
Initial therapy
Methotrexate is now first-line
Sulfasalazine
Current indication for anti-TNF drugs
When inadequate response to at least two DMARDs including methotrexate
TNF-alpha blockers
Etanercept: s/c administration, can cause demyelination
Infliximab: IV administration, risks include reactivation of TB
Rituximab anti-CD20 monoclonal antibody, results in B-cell depletion
Question 58
A 64-year-old female is referred to rheumatology out-patients by her GP with a history of arthritis in both
hands. Which one of the following x-ray findings would most favour a diagnosis of rheumatoid arthritis
over other possible causes?
A. Loss of joint space
B. Periarticular osteopenia
C. Subchondral sclerosis
D. Osteophytes
E. Subchondral cysts
Periarticular osteopenia and osteoporosis would point towards diagnosis of

rheumatoid arthritis (RA). Loss of joint space is common in both RA and osteoarthritis
Rheumatoid arthritis:
Early x-ray findings
Loss of joint space
Juxta-articular osteoporosis
Soft-tissue swelling
Late x-ray findings
Periarticular erosions
Subluxation
Question 59
A 40-year-old woman who is known to have systemic lupus erythematosus is reviewed with an
exacerbation of wrist pain. Which one of the following is the most useful marker for monitoring disease
activity?
A. C-reactive protein
B. C2 levels
C. Anti-nuclear antibody titres
D. Anti-dsDNA titres
E. Anti-Sm titres
SLE: Immunology
Anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
Anti-Smith: most specific (> 99%), sensitivity (30%)
Monitoring
ESR: during active disease the CRP is characteristically normal - a raised CRP may indicate underlying
infection
Complement levels (C3, C4) are low during active disease (formation of complexes leads to consumption
of complement)
Anti-dsDNA titres can be used for disease

monitoring (but note not present in all patients)
Question 60
A 39-year-old woman with a history of rheumatoid arthritis presents with a two day history of a red right
eye. There is no itch or pain. Pupils are 3mm, equal and reactive to light. Visual acuity is 6/5 in both eyes.
A. Keratoconjunctivitis sicca
B. Scleritis
C. Glaucoma
D. Episcleritis
E. Anterior uveitis
Scleritis is painful, episcleritis is not painful

Rheumatoid arthritis:
Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having eye problems
Ocular manifestations
Keratoconjunctivitis sicca (most common)
Episcleritis (erythema)
Scleritis (erythema and pain)
Corneal ulceration
Keratitis
Iatrogenic
Steroid-induced cataracts
Chloroquine retinopathy
Question 61
A 69-year-old man presents with an acute episode of gout on his left first metatarsal-phalangeal joint.
What is the most likely underlying mechanism?
A. Sedentary lifestyle
B. Decreased renal excretion of uric acid
C. Increased endogenous production of uric acid
D. Starvation
E. Too much protein in diet
The vast majority of gout is due to

decreased renal excretion of uric acid
Decreased renal excretion of uric acid is thought to account for 90% of cases of primary gout. Secondary
risk factors such as alcohol intake and medications should also be investigated
Decreased excretion of uric acid
Drugs*: diuretics
Renal failure
Lead toxicity
Increased production of uric acid
Myeloproliferative/lymphoproliferative disorder
Cytotoxic drugs
Severe psoriasis
Lesch-Nyhan syndrome hypoxanthine-guanine phosphoribosyl transferase deficiency
Inheritance = x-linked recessive
Features: gout, renal failure, learning difficulties, head-banging
*aspirin in a dose of 75-150mg is not thought to have a significant effect on plasma urate levels - the
British Society for Rheumatology recommend it should be continued if required for cardiovascular
prophylaxis
Question 62
Which one of the following conditions has polygenic inheritance? A.S.

A. Bartter's syndrome
B. Huntington disease
C. Ankylosing spondylitis
D. Fragile X syndrome
E. Von Willebrand's disease
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathies. It typically presents in males (sex
ratio 5:1) aged 20-30 years old
HLA-B27 is of little use in making the diagnosis as it is positive in:
90% of patients with ankylosing spondylitis
10% of normal patients
Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis
Management (NICE in 2008)
NSAIDs
Physiotherapy
Sulphasalazine may be useful if there is peripheral joint involvement - doesn't improve spinal mobility
TNF-alpha blockers such as etanercept and adalimumab are increasingly used.
Question 63
A 34-year-old is diagnosed with chronic fatigue syndrome. Which one of the following interventions is
least useful?
A. Advice to go to the gym
B. Cognitive behaviour therapy
C. Referral to a pain management clinic if pain is a predominant feature
D. Advice about pacing of activities
E. Low-dose amitriptyline for poor sleep
Chronic fatigue syndrome

Diagnosed after at least 4 months of disabling fatigue affecting mental and physical function more than
50% of the time in the absence of other disease which may explain symptoms
Epidemiology
More common in females
Past psychiatric history has not been shown to be a risk factor
Features include
- Fatigue is the central feature
– sleep problems, such as insomnia, hypersomnia, unrefreshing sleep, a disturbed sleep–wake cycle
– Muscle and/or joint pains
– Headaches
– Painful lymph nodes without enlargement
– Sore throat
– Cognitive dysfunction, such as difficulty thinking, inability to concentrate, impairment of short-term
memory, and difficulties with word-finding
– Physical or mental exertion makes symptoms worse
– General malaise or ‘flu-like’ symptoms
– Dizziness
- Nausea
– Palpitations
Investigation
NICE guidelines suggest carrying out a large number of screening blood tests to exclude other pathology
e.g. FBC, U&E, LFT, glucose, TFT, ESR, CRP, calcium, CK, ferritin*, coeliac screening and also
urinalysis
Management
- Cognitive behaviour therapy - very effective, number needed to treat = 2
- Graded exercise therapy - a formal supervised program, not advice to go to the gym
- 'Pacing' - organising activities to avoid tiring
- Low-dose amitriptyline may be useful for poor sleep
- Referral to a pain management clinic if pain is a predominant feature
Better prognosis in children
*children and young people only
Question 64
A 54-year-old farm worker presents for review. She has recently been diagnosed with osteoarthritis of the
hand but has no other past medical history of note. Despite regular paracetamol she is still experiencing
considerable pain, especially around the base of both thumbs. What is the most suitable next management
step?
A. Add oral diclofenac + lansoprazole
B. Switch paracetamol for co-codamol 8/500
C. Add topical ibuprofen
D. Add oral ibuprofenia
E. Add oral glucosamine
Osteoarthritis - paracetamol + topical NSAIDs (if knee/hand) first-line

- All patients should be offered help with weight loss, given advice about local muscle strengthening
- Paracetamol and topical NSAIDs are first-line analgesics. Topical NSAIDs are indicated only for OA of
the knee or hand
- Second-line treatment is oral NSAIDs/COX-2 inhibitors, opioids, capsaicin cream and intra-articular
- Non-pharmacological treatment options include supports and braces, TENS and shock absorbing insoles
or shoes
- If conservative methods fail then refer for consideration of joint replacement
Question 65
A 54-year-old man is recovering following his first episode of gout. The pain and inflammation settled 4
days ago. He has no risk factors for the development of gout and there is no evidence of gouty tophi on
examination. What is the most suitable point to start uric acid lowering therapy?
A. Immediately
B. If more than 6 episodes of gout in a 1 year period
C. If one further attack of gout in the next 12 months
D. 4 weeks after the initial attack of gout has settled
E. If more than 4 episodes of gout in a 1 year period
Gout: start allopurinol if >= 2 attacks in 12 month period

Acute management
NSAIDs
Allopurinol prophylaxis:-
Allopurinol should not be started until 2 weeks after an acute attack has settled
Initial dose of 100 mg od, with the dose titrated every few weeks to aim for a
Serum uric acid of < 300 µmol/l
NSAID or colchicine cover should be used when starting allopurinol
lowering drug therapy should be started if a second attack or further attacks occur within 1 year'
Tophi
Renal disease
Question 66
A 63-year-old man presents to the Emergency Department with a 2 day history of a painful and swollen
left knee joint. Aspiration reveals positively birefringent crystals and no organisms are seen. Which of the
following conditions are not recognised causes of the underlying condition?
A. Haemochromatosis
B. Low magnesium
C. High phosphate
D. Acromegaly
E. Hyperparathyroidism
A low phosphate predisposes to pseudogout

Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate
dihydrate in the synovium
Features
Knee, wrist and shoulders most commonly affected
X-ray: chondrocalcinosis
Risk factors
Hyperparathyroidism
Hypothyroidism
Haemochromatosis
Acromegaly
Low magnesium, low phosphate
Wilson's disease
Management
Aspiration of joint fluid, to exclude septic arthritis and show weakly-positively birefringent brick shaped
crystals
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
Question 67
Which one of the following would not suggest an underlying connective tissue disorder in a patient with
Raynaud's?
A. Unilateral symptoms
B. Digital ulcers
C. Presence of autoantibodies
D. Onset at 25 years old
E. Calcinosis
Raynaud's disease (i.e. primary) presents in young women with bilateral symptoms
Unilateral symptoms
Rashes
Secondary causes
Leukaemia
Cervical rib
Management
Question 68
Which one of the following is most likely to indicate an underlying connective tissue disorder in a patient
with Raynaud's phenomenon?
A. Chilblains
B. Bilateral symptoms
C. Female patient
D. Onset at 18 years old
E. Recurrent miscarriages
Raynaud's disease (i.e. primary) presents in young women with bilateral symptoms
A history of recurrent miscarriages could indicate systemic lupus erythematous or anti-phospholipid
syndrome. Chilblains (pernio) are itchy, painful purple swellings which occur on the fingers and toes after
exposure to the cold. They are occasionally associated with underlying connective tissue disease but this is
rare
Unilateral symptoms
Rashes
Secondary causes
Leukaemia
Cervical rib
Management
Question 69
Which of the following statements is true regarding psoriatic arthropathy?
A. Skin disease always precedes joint disease
B. Approximately one-third of patients with psoriasis eventually develop arthropathy
C. The mainstay of management is analgesia, physiotherapy and joint replacement
D. Males and females are equally affected
E. Arthritis mutilans is the most common subtype
Males and females are affected equally by psoriatic arthritis

Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the development of
skin lesions. Around 10% percent of patients with skin lesions develop an arthropathy with males and
females being equally affected
Types*
Rheumatoid-like polyarthritis: (30-40%, most common type)
Asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
Sacroilitis
DIP joint disease (10%)
Arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')
Management
Treat as rheumatoid arthritis but better prognosis
*Until recently it was thought asymmetrical oligoarthritis was the most common type, based on data from
the original 1973 Moll and Wright paper.
Question 70
A 33-year-old female is admitted to the Emergency Department due to right-sided weakness. She has a
past history of deep vein thrombosis following the birth of her daughter. The only other past history of note
is two miscarriages. A CT head confirms an ischaemic stroke in the left middle cerebral artery territory.
What is the likely finding on echocardiography?
A. Normal
B. Dilated cardiomyopathy
C. Bicuspid aortic valve
D. Atrial septal defect
E. Ventricular septal defect
This is a typical MRCP question. On first sight this question appears to be pointing towards a paradoxical
embolus. However, given the history of miscarriages a diagnosis of antiphospholipid syndrome is more
likely
Features
Livedo reticularis
Thrombocytopenia
Prolonged APTT
- Initial venous thromboembolic events: evidence currently supports use of warfarin with a target INR of 2-
3 for 6 months
- Recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then
increase target INR to 3-4
- Arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
Question 71
A 71-year-old man presents with an erythematous, swollen first metatarsophalangeal joint on the left foot.
This is causing him considerable pain and he is having difficulty walking. He has never had any previous
similar episodes. His past medical history includes atrial fibrillation and type 2 diabetes mellitus and his
current medications are warfarin, metformin and simvastatin. What is the most appropriate treatment of
this episode?
A.Intra-articular corticosteroid
B.Colchicine
C.Ibuprofen
D.Diclofenac
E.Prednisolone
NSAIDs should be avoided in elderly patients taking warfarin due to the risk of a life-threatening
gastrointestinal haemorrhage. Oral steroids are an option but would upset his diabetic control.
Acute management
NSAIDs
Allopurinol prophylaxis -
Allopurinol should not be started until 2 weeks after an acute attack has settled initial dose of 100 mg od,
with the dose titrated every few weeks to aim for aserum uric acid of < 300 µmol/l
NSAID or colchicine cover should be used when starting allopurinol
lowering drug therapy should be started if asecond attack or further attacks occur within 1 year'
Tophi
Renal disease
Question 72
A 66-year-old female presents with pain at the base of her left thumb. She has no past medical history of
note. On examination there is diffuse tenderness and swelling of her left first carpometacarpal joint. What
is the most likely diagnosis?
A. Osteoarthrit
B. De Quervain's tenosynovitis
C. Gout
D. Rheumatoid arthritis
E. Primary hyperparathyroidism
The trapeziometacarpal joint (base of thumb) is the most common site of hand osteoarthritis
All patients should be offered help with weight loss, given advice about local muscle strengthening
Paracetamol and topical NSAIDs are first-line analgesics. Topical NSAIDs are indicated only for OA of
the knee or hand
Second-line treatment is oral NSAIDs/COX-2 inhibitors, opioids, capsaicin cream and intra-articular
Non-pharmacological treatment options include supports and braces, TENS and shock absorbing insoles or
shoes
If conservative methods fail then refer for consideration of joint replacement
Question 73
A 33-year-old female presents 6 weeks after the birth of her first child with a two-week history of
polyarthralgia, fever and a skin rash. First-line investigations show: ESR 45 mm/hour
A. Polymorphic eruption of pregnancy
B. Systemic lupus erythematous
C. Rheumatoid arthritis
D. Reactive arthritis
E. Pseudogout
Unlike many autoimmune diseases systemic lupus erythematous (SLE) often becomes worse during
pregnancy and the puerperium
SLE: pregnancy overview
Risk of maternal autoantibodies crossing placenta leads to condition termed neonatal lupus erythematous
Neonatal complications include congenital heart block
Strongly associated with anti-Ro (SSA) antibodies
Question 74
A 57-year-old woman with a history of polymyalgia rheumatica has been taking prednisolone 10 mg for
the past 5 months. A DEXA scan is reported as follows:
L2 T-score- 1.6 SD
Femoral neck T-score- 1.7 SD
What is the most suitable management?
A. No treatment
B. Vitamin D + calcium supplementation + repeat DEXA scan in 6 months
C. Vitamin D + calcium supplementation
D. Vitamin D + calcium supplementation + hormone replacement therapy
E. Vitamin D + calcium supplementation + oral bisphosphonate
Osteoporosis: glucocorticoid-induced
Patients who take the equivalent of prednisolone 7.5 mg or more each day for 3 months or longer should be
assessed and where necessary given prophylactic treatment
Assessment for treatment - patients taking the equivalent of prednisolone 7.5 mg or more each day for 3
months, and one of the following
- Are over the age of 65 years
- Have a history of a fragility fracture
- Have a T-score less than - 1.5 SD
Treatment
First-line: oral bisphosphonate
Second-line: alfacalcidol or calcitriol
Question 75
A 73-year-old man presents pain in his right thigh. This has been getting progressively worse for the past 9
months despite being otherwise well. An x-ray is reported as follows:
X-ray right femurRadiolucency of subarticular region suggestive of osteolysis. Some areas of patchy
sclerosis. Bloods tests show:
Calcium 2.38 mmol/l
Phosphate 0.85 mmol/l
Alkaline phosphatase 544 u/L
Prostate specific antigen 4.4 ng/ml
What is the most appropriate action?
A. Vitamin D supplementation
B. Check serum testosterone
C. Referral to an orthopaedic surgeon
D. Referral to a urologist
E. IV bisphosphonates
This patient has Paget's disease as evidenced by an isolated rise in ALP and characteristic x-ray changes.
As he has bone pain he should be treated with bisphosphonates. A PSA of 4.4 ng/ml is probably normal in
a 73-year-old man and is certainly not consistent with metastatic prostate cancer
Paget's disease of the bone
Paget's disease is a disease of increased but uncontrolled bone turnover. It is thought to be primarily a
disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity.
Paget's disease is common (UK prevalence 5%) but symptomatic in only 1 in 20 patients
Increasing age
Male sex
Northern latitude
Family history
Clinical features - only 5% of patients are symptomatic
Bone pain (e.g. pelvis, lumbar spine, and femur)
Classical, untreated features: bowing of tibia, bossing of skull
Raised alkaline phosphatase (ALP) - calcium* and phosphate are typically normal
Skull x-ray: thickened vault, osteoporosis circumscripta
Indications for treatment include bone pain, skull or long bone deformity, fracture, periarticular Paget's
Bisphosphonate (either oral risedronate or IV zoledronate)
Calcitonin is less commonly used now
Complications
Deafness (cranial nerve entrapment)
Bone sarcoma (1% if affected for > 10 years)
Fractures
Skull thickening
High-output cardiac failure
*usually normal in this condition but hypercalcaemia may occur with prolonged immobilisation
Question 76
Which one of the following is most recognised as a risk factor for developing osteoporosis?
A. Osteogenesis imperfecta
B. Marfan's syndrome
C. Myotonic dystrophy
D. Duchenne muscular dystrophy
E. Ehler-Danlos syndrome

Family history
Female sex
Increasing age
Deficient diet
Sedentary lifestyle
Smoking
Premature menopause
Low body weight

Endocrine: glucocorticoid excess (e.g. Cushing's, steroid therapy), hyperthyroidism, hypogonadism (e.g.
Turner's), growth hormone deficiency, hyperparathyroidism, diabetes mellitus
Question 77
A 66-year-old female is on long-term prednisolone therapy for polymyalgia rheumatica. What is the most
appropriate protection against osteoporosis?
A. Hormone replacement therapy
B. Calcitonin
C. Oral bisphosphonate
D. Calcium and vitamin D
E. Hip-protectors
Oral bisphosphonate therapy is recommended for patients older than 65 years who have taken, or who are
likely to remain on oral corticosteroids for more than 3 months
Bisphosphonates
Bisphosphonates are analogues of pyrophosphate, a molecule which decreases demineralisation in bone.
They inhibit osteoclasts by reducing recruitment and promoting apoptosis
Clinical uses
Prevention and treatment of osteoporosis
Hypercalcaemia
Paget's disease
Pain from bone metatases
Adverse effects
Oesophageal reactions: oesophagitis, oesophageal ulcers (especially alendronate)
Osteonecrosis of the jaw
The BNF suggests the following counselling for patients taking oral bisphosphonates
'Tablets should be swallowed whole with plenty of water while sitting or standing; to be given on an empty
stomach at least 30 minutes before breakfast (or another oral medication); patient should stand or sit
upright for at least 30 minutes after taking tablet'
Question 78
A 54-year-old woman is reviewed. She was discharged from the psychiatric ward around 5 weeks ago
following an admission for an acute psychotic episode. Her psychotic symptoms have settled on
risperidone but unfortunately she has now developed a dry mouth and arthralgia in both hands. A number
of blood tests are requested:
Rheumatoid factor Positive
Anti-Ro Positive
Anti-Smith Negative
ANA Positive
C4 Low

B. Sarcoidosis
C. Drug-induced lupus erythematous
D. Rheumatoid arthritis
E. Sjogren's syndrome
Risperidone (unlike chlorpromazine) is not a common trigger of lupus. Anti-Ro is positive in around 70%
of patients with Sjogren's syndrome compared to around 10% of patients with rheumatoid arthritis
Sjogren's syndrome
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.
It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it
usually develops around 10 years after the initial onset. Sjogren's syndrome is much more common in
females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold)
Features
Dry mouth
Vaginal dryness
Arthralgia
Raynaud's, myalgia
Investigation
ANA positive in 70%
Schirmer's test: filter paper near conjunctival sac to measure tears formation
Management
Question 79
Which one of the following is most recognised as a potential complication in a patient with ankylosing
spondylitis?
A. Heart block
B. Aortic stenosis
C. Achalasia
D. Diabetes mellitus
E. Bronchiectasis
Ankylosing spondylitis: Features

Typically a young man who presents with lower back pain and stiffness
Stiffness is usually worse in morning and improves with activity
Peripheral arthritis (25%, more common if female)
Other features - the 'A's

Apical fibrosis
Anterior uveitis
Achilles tendonitis
AV node block
Amyloidosis
And cauda equina syndrome
X-rays are often normal early in disease, later changes include:
Sacroilitis: subchondral erosions, sclerosis
Squaring of lumbar vertebrae
'Bamboo spine' (late & uncommon)
- Chest x-ray: apical fibrosis
Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and
ankylosis of the costovertebral joints
Question 80
Which one of the following statements is true regarding cytoplasmic anti-neutrophil cytoplasmic
antibodies (cANCA)?
A. Targeted against myeloperoxidase
B. Associated with Wegener's granulomatosis
C. Can be used to monitor activity in autoimmune haemolytic anaemia
D. Is more commonly seen in ulcerative colitis than perinuclear ANCA
E. Positive in > 90% of hepatitis C associated vasculitis
cANCA = Wegener's; pANCA = Churg-Strauss + others

ANCA
There are two main types of anti-neutrophil cytoplasmic antibodies (ANCA) - cytoplasmic (cANCA) and
perinuclear (pANCA)
For the exam, remember:
cANCA - Wegener's granulomatosis
pANCA - Churg-Strauss syndrome + others (see below)
cANCA
Most common target serine proteinase 3 (PR3)
Some correlation between cANCA levels and disease activity
Wegener's granulomatosis, positive in > 90%
Microscopic polyangiitis, positive in 40%
pANCA
Most common target is myeloperoxidase (MPO)
Cannot use level of pANCA to monitor disease activity
Associated with immune crescentic glomerulonephritis (positive in c. 80% of patients)
Microscopic polyangiitis, positive in 50-75%
Churg-Strauss syndrome, positive in 60%
Wegener's granulomatosis, positive in 25%
Other causes of positive ANCA (usually pANCA)
Inflammatory bowel disease (UC > Crohn's)
Connective tissue disorders: RA, SLE, Sjogren's
Autoimmune hepatitis
Question 81
Which one of the following antibodies is most specific for systemic lupus erythematous?
A. Anti-neutrophil cytoplasmic antibodies
B. Anti-nuclear antibodies
C. Anti-Sm antibodies
D. Anti-dsDNA antibodies
E. Anti-cardiolipin antibodies
SLE: ANA is 99% sensitive - anti-Sm & anti-dsDNA are 99% specific
This is tough as both anti-Sm and anti-dsDNA antibodies are highly specific for SLE. However, anti-Sm
antibodies are slightly more specific
SLE: investigations
Immunology
Anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
Anti-Smith: most specific (> 99%), sensitivity (30%)
Monitoring
ESR: during active disease the CRP is characteristically normal - a raised CRP may indicate underlying
infection
Complement levels (C3, C4) are low during active disease (formation of complexes leads to consumption
of complement)
Anti-dsDNA titres can be used for disease monitoring (but note not present in all patients)
Question 82
A 62-year-old man with lung cancer is suspected of having dermatomyositis. Which one of the following
antibodies is most likely to be positive?
A. Anti-nuclear antibodies
B. Anti-centromere bodies
C. Anti-scl-70 antibodies
D. Anti-Jo-1 antibodies
E. Anti-Mi-2 antibodies
Dermatomyositis: Investigations
Elevated creatine kinase
EMG
Muscle biopsy
Anti-Jo-1 antibodies are not commonly seen in dermatomyositis - they are more common in polymyositis
where they are seen in a pattern of disease associated with lung involvement, Raynaud's and fever
ANA positive in 60%
Management
Prednisolone
Question 83
Which one of the following is most recognised as a risk factor for developing osteoporosis?
A. Cystinuria
B. Psoriasis
C. Cystinosis
D. Homocystinuria
E. Lichen planus
Family history
Female sex
Increasing age
Deficient diet
Sedentary lifestyle
Smoking
Premature menopause
Low body weight
Endocrine: glucocorticoid excess (e.g. Cushing's, steroid therapy), hyperthyroidism, hypogonadism (e.g.
Turner's), growth hormone deficiency, hyperparathyroidism, diabetes mellitus
Question 84
A 52-year-old man with a history of hepatitis B infection presents with fever and polyarthralgia associated
with a right foot drop. Examination reveals a blood pressure of 180/100 mmHg. What is the most likely
diagnosis?
A. Churg-Straus syndrome
B. Hepatocellular carcinoma
C. Systemic lupus erythematous
D. Polyarteritis nodosa
E. Giant cell arteritis
Polyarteritis nodosa
Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation
leading to aneurysm formation. PAN is more common in middle-aged men and is associated with hepatitis
B infection
Features
Fever, malaise, arthralgia
Hypertension
Mononeuritis multiplex, sensorimotor polyneuropathy
Haematuria, renal failure
Testicular pain
Abdominal pain (e.g. from mesenteric ischaemia)
Perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with
'classic' PAN
Question 85
A 24-year-old female is reviewed in the medical clinic with lethargy and dizzy spells. On examination she
is noted to have an absent left radial pulse. Blood tests are as follows:
Na+ 136 mmol/l
K+ 4.1 mmol/l
Urea 2.3 mmol/l
Creatinine 77 µmol/l
ESR 66 mm/hr
A. Turner's syndrome
B. Takayasu's arteritis
C. Kawasaki disease
D. Coarctation of the aorta
E. Breast carcinoma with local spread
Takayasu's arteritis
Takayasu's arteritis is a large vessel vasculitis. It typically causes occlusion of the aorta and questions
commonly refer to an absent limb pulse. It is more common in females and Asian people
Associations
Renal artery stenosis
Management
Steroids
Question 86
A 47-year-old female is referred to the rheumatology clinic due to cold fingers. Which connective tissue
disease is most strongly associated with Raynaud's phenomenon?
B. Rheumatoid arthritis
C. Systemic sclerosis
D. Sjogren's syndrome
E. Polyarteritis nodosa
Raynaud's phenomenon is associated with all the above conditions but is most strongly linked to systemic
sclerosis. Around 2% of women and 6% of men with Raynaud's phenomenon develop systemic sclerosis
Unilateral symptoms
Rashes
Secondary causes
Leukaemia
Cervical rib
Management
Question 87
In which one of the following conditions is intravenous immunoglobulin therapy most likely to be
beneficial?
A. Graves' ophthalmopathy
B. Kawasaki disease
C. Inclusion body myositis
D. Multiple sclerosis
E. Rheumatoid arthritis
The Department of Health issued guidelines on the use of intravenous immunoglobulins in May 2008
Uses
Primary and secondary immunodeficiency
Idiopathic thrombocytopenic purpura
Myasthenia gravis
Guillain-Barre syndrome
Kawasaki disease
Toxic epidermal necrolysis
Pneumonitis induced by CMV following transplantation
Low serum IgG levels following haematopoietic stem cell transplant for
Malignancy
Dermatomyositis
Chronic inflammatory demyelinating polyradiculopathy
Basics
Formed from large pool of donors (e.g. 5,000)
IgG molecules with a subclass distribution similar to that of normal blood
Half-life of 3 weeks
Question 88
A 43-year-old man with a history of chronic back pain presents complaining of pain in his left eye and
photophobia. On examination the pupil is small, oval shaped and associated with ciliary congestion. What
is the most likely diagnosis?
A. Anterior uveitis
B. Conjunctivitis
C. Scleritis
D. Meningitis
E. Acute angle closure glaucoma
Red eye - glaucoma or uveitis?

Glaucoma: severe pain, haloes, 'semi-dilated' pupil
Uveitis: small, fixed oval pupil, ciliary flush
His chronic back pain may be HLA-B27 related, which is associated with anterior uveitis
Red eye
There are many possible causes of a red eye. It is important to be able to recognise the causes which
require urgent referral to an ophthalmologist. Below is a brief summary of the key distinguishing features
Acute angle closure glaucoma
Severe pain (may be ocular or headache)
Decreased visual acuity, patient sees haloes
Semi-dilated pupil
Hazy cornea
Anterior uveitis
Acute onset
Pain
Blurred vision and photophobia
Small, fixed oval pupil, ciliary flush
Scleritis
Severe pain (may be worse on movement) and tenderness
May be underlying autoimmune disease e.g. rheumatoid arthritis
Conjunctivitis
Purulent discharge if bacterial, clear discharge if viral
Subconjunctival haemorrhage
History of trauma or coughing bouts
Question 89
Which one of the following respiratory disorders is least associated with rheumatoid arthritis?
A. Exudative pleural effusions
B. Emphysema
C. Caplan's syndrome
D. Lower zone pulmonary fibrosis
E. Bronchiolitis obliterans
Rheumatoid arthritis: pulmonary manifestations

A variety of respiratory problems may be seen in patients with rheumatoid arthritis:
Pulmonary fibrosis
Pleural effusion
Pulmonary nodules
Bronchiolitis obliterans
Complications of drug therapy e.g. methotrexate pneumonitis
Pleurisy
Caplan's syndrome - massive fibrotic nodules with occupational coal dust exposure
Infection (possibly atypical) secondary to immunosuppression
Question 90
Which of the following have not been shown to be a marker of poor prognosis in rheumatoid arthritis?
A. Insidious onset
B. Early erosions on x-ray
C. Male sex
D. Rheumatoid nodules
E. Rheumatoid factor positive
Rheumatoid arthritis: prognostic features

Poor prognostic features
Poor functional status at presentation
HLA DR4
X-ray: early erosions (e.g. after < 2 years)
Extra articular features e.g. nodules
Female sex
Insidious onset
Anti-CCP antibodies
Question 91
Churg-Strauss syndrome is associated with each one of the following, except:ia
A. Asthma
B. Lymphocytosis
C. Paranasal sinusitis
D. Mononeuritis multiplex
E. pANCA
Churg-Strauss syndrome is an ANCA associated small-medium vessel vasculitis

Features
asthma
blood eosinophilia (e.g. > 10%)
paranasal sinusitis
mononeuritis multiplex
pANCA positive in 60%
Leukotriene receptor antagonists may precipitate the disease
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Question 1

Septic arthritis - most common organism: Staphylococcus aureus

Septic arthritis overview

SLE: C3 & C4 low

Oral ulcers + genital ulcers + anterior uveitis = Behcet's

What is the most likely diagnosis?

Adult onset Still's disease is typically rheumatoid factor negative

What is the most likely diagnosis?

Hepatitis C infection is associated with type II (mixed) cryoglobulinaemia, suggested by

Peak onset = 30-50 years, although occurs in all age groups

Thiopurine methyltransferase (TPMT) deficiency is present in c. 1 in 300 people and predisposes to

Systemic lupus erythematosus

Osteoarthritis - paracetamol + topical NSAIDs (if knee/hand) first-line

Churg-Strauss syndrome, positive in 60%

Rheumatoid arthritis poor prognostic features:-

Urethritis + arthritis + conjunctivitis = reactive arthritis

Rheumatoid arthritis - TNF is key in pathophysiology

Adverse effects of TNF blockers include reactivation of latent tuberculosis and

Lateral epicondylitis: worse on resisted wrist extension/suppination whilst elbow extended

3SSS (6 wk.), R2X

Diffuse cutaneous systemic sclerosis

Mixed connective tissue disease

Diabetic amyotrophy affects the lower limbs

SLE: pregnancy overview

Perinuclear-antineutrophil cytoplasmic antibodies are found in around 20% of patients

Management of fibromyalgia may consist of the following:

HLA-B27 is of little use in making the diagnosis as it is positive in:

Management (NICE in 2008)

Discoid lupus erythematosus is characterised by follicular keratin plugs

Discoid lupus erythematous is a benign disorder generally seen in younger

Osteoporosis: Risk factors

Lithium was actually used to treat gout in the 19th century

Ergotamine rather than pizotifen is associated with Raynaud's phenomenon

Drugs: oral contraceptive pill, ergot

Anti-Jo-1 antibodies are more

Periarticular osteopenia and osteoporosis would point towards diagnosis of

Anti-dsDNA titres can be used for disease

Scleritis is painful, episcleritis is not painful

The vast majority of gout is due to

Which one of the following conditions has polygenic inheritance? A.S.

Chronic fatigue syndrome

Osteoarthritis - paracetamol + topical NSAIDs (if knee/hand) first-line

Gout: start allopurinol if >= 2 attacks in 12 month period

A low phosphate predisposes to pseudogout

Males and females are affected equally by psoriatic arthritis

Osteoporosis: Risk factors

Diseases which predispose

What is the most likely diagnosis?

Ankylosing spondylitis: Features

Other features - the 'A's

cANCA = Wegener's; pANCA = Churg-Strauss + others

Red eye - glaucoma or uveitis?

Rheumatoid arthritis: pulmonary manifestations

Rheumatoid arthritis: prognostic features

Churg-Strauss syndrome is an ANCA associated small-medium vessel vasculitis

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