Rheumatology

Ankylosing spondylitis
➢ An HLA-B27 associated spondyloarthropathy which primarily involves the axial skeleton (i.e. sacroiliitis and
spondylitis)
➢ It’s classified as a seronegative spondyloarthropathy, a group of diseases that are negative for rheumatoid
factor
➢ Common in patients with IBD
Features
• Young man (<30 years old) presenting with lower back
pain and stiffness
• Stiffness which is worse in the morning and improves
with exercise
• A strong association with HLA-B27
• There is often tenderness of the sacroiliac joints or a
limited range of spinal motion
Examination
• Schober's test
- A line is drawn 10 cm above and another line 5 cm
below the back dimples (dimples of Venus)
- The distance between the two lines should increase by more than 5 cm when the patient bends as far
forward as possible
Other important features
- Anterior uveitis (20-30%) → presents with an acutely painful red eye
and severe photophobia
- Aortic regurgitation
Investigations
• Plain x-ray of the sacroiliac joints → This is the most useful
investigation
- It would show evidence of sacroiliitis which is the earliest finding
- Later findings once there is significant chronic spine inflammation
include a “bamboo spine” and squaring of the vertebral bodies
• MRI → more sensitive in demonstrating sacroiliitis
• DO NOT use HLA-B27 to make the diagnosis as it is also positive in 10%
of normal patients
• Elevated ESR and CRP
• FBC → normochromic normocytic anemia
Management
- First line → NSAIDS
- Second line → Anti-TNF therapy

PEPSI

P → Pain lower back

E → Eye symptoms (anterior uveitis)
P → Progressive loss of special movements
S → Stiffness
I → IBD

PLABverse - plabverse@yahoo.com 1
 Rheumatology
Gout
➢ A disease that affects middle-aged men and presents most commonly with acute Monoarthritis
➢ The metatarsophalangeal joint of the first toe is commonly affected (podagra), but other joints like the knee,
ankle, PIPs, or DIPs may be initially involved
➢ The first episode commonly occurs at night with severe joint pain waking the patient from sleep
➢ The joint rapidly becomes warm, red, and tender (it looks exactly like cellulitis). Without treatment the joint
pain goes away spontaneously in 2 weeks
➢ Certain events that precipitate gout sometimes precede the attack → a person has consumed excessive
amounts of alcohol or started taking diuretics such as thiazide diuretics (xipamide, metolazone, indapamide)
or furosemide → could be indirectly stated by mentioning a cardiac patient
➢ Gout can be associated with: PRV, CML, TL$, Psoriasis
Diagnosis
• Diagnosis is made by → Joint aspirate for microscopy → MSU crystals
• MSU crystals deposit → Tophi
• The serum uric acid during the acute attack may be normal or low and shouldn’t be measured until 4 weeks
after an acute attack → no value in the diagnosis of acute urate arthropathy
Treatment
• Acute management
- NSAIDs (naproxen) → 1st line unless the patient has asthma or renal insufficiency, often prescribed with
PPIs for gastric protection and in elderly
- Colchicine (SE → diarrhea and nausea), should be avoided in renal insufficiency as well
- Intra-articular steroid injection, could be given as tablets or IM, prescribed if asthma or renal insufficiency
• Chronic hypouricemic therapy
- Allopurinol should not be started until 2 weeks after an acute attack has settled as it may precipitate a
further attack if started too early, used to prevent recurrence not to treat
- NSAID or colchicine cover should be used when starting allopurinol

Drugs that precipitate gout [FACT] + Niacin

F → Furosemide
A → Alcohol
C → Cytotoxic drugs/cyclosporine
T → Thiazide

Pseudogout
➢ Usually involves larger joints (knees and ankles)
➢ There should be a history of hemochromatosis or hyperparathyroidism
Diagnosis
• Joint aspirate → calcium pyrophosphate (rhomboid crystals), +ve birefringence

Rheumatoid arthritis
Management
• Acute → NSAIDs (ibuprofen, naproxen), PPIs are used to prevent GI bleed
• Long-term → DMARDs (methotrexate, hydroxychloroquine, sulfasalazine)

PLABverse - plabverse@yahoo.com 2
 Rheumatology
Septic arthritis
➢ The two most common organisms:
- Staphylococcus Aureus → Most common pathogen for septic arthritis overall
- Neisseria gonorrhoeae → Seen in young, sexually active adults
➢ Mode of infection:
- Hematogenous spread during transient bacteremia
- Through a skin lesion that penetrates the joint
- Local spread from a contiguous infected site
Knee is involved in more than 50% of cases
Risk factors
• Prior joint damage (rheumatoid arthritis, gout, osteoarthritis)
• Immunodeficiency states (HIV, corticosteroid use)
• Diabetes
Presentation
• Single swollen, red joint with pain on active or passive movement
• Restricted joint movement
• Fever and rigors ➢ Remember this triad of fever, pain and impaired range of motion
Investigations
• Aspiration of synovial fluid → Sent for gram staining, leukocyte count, microscopy and culture
• Blood cultures → Remember most cases of septic arthritis are due to hematogenous spread
Management
• Flucloxacillin for 4 to 6 weeks → IV antibiotics should be commenced before culture’s results
• If penicillin-allergic → Use clindamycin
• If gonococcal arthritis (NG) → Use cefotaxime or ceftriaxone
• If infection not responding to antibiotics → Perform repeated percutaneous aspiration
In general, intravenous antibiotics are used for 7 days until the swelling subsides and blood cultures become
negative. This is followed by a 4-week course of oral antibiotics

Reactive arthritis
➢ A form of seronegative spondyloarthritis clinically associated with back pain, migratory oligoarthritis
(affecting two to four joints during the first six months of disease) and extra-articular symptoms
➢ Typically follow a GI (campylobacter, salmonella, shigella) or urogenital infection (chlamydia trachomatis)
➢ The initial infection may be so mild it goes unnoticed
➢ Unlike septic arthritis, fever is not a typical feature however it can be seen
Features
• Young adults
• Develops 2-4 weeks after an initial infection which have been sexually acquired or gastrointestinal in origin
• Asymmetrical, especially lower extremity, oligoarhtritis in the major symptom (usually knees and ankles)
• Reiter triad [can’t see, can’t pee, can’t climb a tree]→ conjunctivitis/anterior uveitis + urethritis + arthritis
• Skin
- Circinate balanitis → painless vesicles on the coronal margin of the prepuce (foreskin)
- Keratoderma blennorrhagicum → waxy yellow/brown maculopapular rash seen on palms and soles
- Erythema nodosum → tender red nodules on the shins
Treatment
• NSAIDs
PLABverse - plabverse@yahoo.com 3
 Rheumatology
Polymyositis
Features
• Muscle weakness involve the proximal muscles: lifting objects, combing hair, getting up from the chair
• Weakness usually symmetric and diffuse, involving the proximal muscles of the neck, shoulders, trunk, hips
and thighs. LL muscles tend to be clinically symptomatic first
• Fatigue, myalgia and muscle cramps
• Advanced cases: dyspnea and dysphagia
Investigation
• Raised CK
• Raised Aldolase level
• Autoantibodies: Anti-Jo-1 antibodies. Note that these are common in patients with polymyositis than in
patients with dermatomyositis
• Muscle biopsy can be diagnostic
Treatment
• Steroids

Polymyositis Polymyalgia rheumatica

• Stiffness not weakness

• Difficulty to raise hands above head
• Weakness of the proximal muscle + raised CK • Fever + weight loss
• Associated with breast/lung cancer • ESR> 30mm/h or CRP> 6mg/mL
• CK is NOT raised
• Associated with Temporal arteritis (TA)

Chronic fatigue syndrome (CFS) or Myalgic Encephalomyelitis (ME)

➢ Severe fatigue + unrelated to exertion or triggered by minimal activity + unrelieved by rest
➢ All tests are normal
➢ Sometimes the symptoms start with a viral infection and it keeps progressing
Features
• Persistent or recurrent fatigue
• Fatigue unexplained by other conditions
• Difficulty sleeping
• Muscle and joint pain at multi-sites without evidence of inflammation
• Painful lymph nodes without pathological enlargement
• Headaches
• Cognitive dysfunction (difficulty thinking, concentrating or finding words)

PLABverse - plabverse@yahoo.com 4
 Rheumatology
Systemic lupus erythematous (SLE)
➢ Whenever patient has multi-system involvement + raised ESR + normal CRP → SLE
Features
• Remitting and relapsing illness
• Mouth ulcers → large, multiple and painful
• Lymphadenopathy
• Malar (butterfly) rash, sparing nasolabial folds
• Discoid rash: scaly, erythematous, well-demarcated rash in sun-exposed areas
• Photosensitivity
Most common drugs causing induced Lupus
• Arthralgia
• Hydralazine
• Raynaud’s phenomenon, occurs in 1/5 of the patients • Isoniazid
• Cardiovascular → pericarditis • Procainamide
• Respiratory → pleurisy, fibrosing alveolitis
• Renal → AGN, often asymptomatic and detected by proteinuria, hypertension or a raised serum urea and
creatinine
• Neuropsychiatric → anxiety and depression
Investigation
• FBC and ESR • Highly sensitive tests are used to screen (initial tests)
- Mild anemia while highly specific tests are used to diagnose
- Raised ESR
• Autoantibodies
- ANA → most sensitive (95%) but not diagnostic in the absence of clinical features
- Anti-dsDNA → highly specific (>99%), but less sensitive (70%)
- Anti-Smith → most specific (>99%), but even less sensitive (30%-40%)
- Anti-histone → drug-induced lupus ANA antibodies are often this type
- Rheumatoid factor → positive in 20%
• Complement levels (C3, C4) → low during active disease (formation of complexes leads to consumption of
complement)

SLE [SOAP BRAIN MD]

S → Serosis (pleuritis, pericarditis)
O → Oral ulcers
A → Arthritis
P → Photosensitivity

B → Blood (all are low; Anemia, Leukopenia, Thrombocytopenia)

R → Renal (AGN)
A → ANA
I → Immunological (Anti-dsDNA)
N → Neurological (psych, seizures)

M → Malar rash
D → Discoid rash

PLABverse - plabverse@yahoo.com 5
 Rheumatology
Churg-Strauss Syndrome
➢ Also called “eosinophilic granulomatosis with polyangiitis” or “allergic granulomatosis”
➢ Rare diffuse vasculitis affecting coronary, pulmonary, cerebral, abdominal visceral and skin circulations
➢ The vasculitis affects small- and medium-sized arteries and veins and is associated with asthma
➢ The cardinal manifestations of Churg-Strauss syndrome are Asthma, Eosinophilia, and Lung involvement
Six criteria (4/6 is diagnostic)
• Asthma (wheezing, expiratory rhonchi)
• Eosinophilia of more than 10% in peripheral blood
• Pulmonary infiltrates (may be transient)
• Paranasal sinusitis
• Histological confirmation of vasculitis with extravascular eosinophils
• Mononeuritis multiplex (peripheral neuropathy)
Presentation
• Pulmonary → asthma
• Upper respiratory tract → allergic rhinitis, paranasal sinusitis, nasal polyposis
• Cardiac involvement is common → heart failure, myocarditis and myocardial infarction
• Skin → purpura, skin nodules
• Renal → glomerulonephritis
• Peripheral neuropathy → mononeuritis multiplex is the most frequent form
Investigations
• P-ANCA → +ve
• FBC → Eosinophilia and anemia
• Elevated ESR and CRP
• Elevated serum creatinine
• Increased serum IgE levels
• CXR → pulmonary opacities, transient pulmonary infiltrates, and bilateral multifocal consolidation
• High-resolution CT → Ground-glass attenuation
• Biopsy → small necrotizing granulomas, as well as necrotizing vasculitis (found especially in the lung)

Granulomatosis with polyangiitis (GPA) or Wegner’s granulomatosis

➢ Idiopathic small to medium vessel vasculitis
➢ Appears around middle-age
➢ A person bleeding from his nose, lungs and kidneys
Features
• URTIs
- Nosebleeds • Churg-Strauss → P-ANCA
- Nasal crusting • Granulomatosis with polyangiitis → C-ANCA
- Chronic sinusitis
• Lungs
- Hemoptysis
- Cough • Jaundice + hemoptysis → alpha 1 anti trypsin deficiency
• Hematuria + hemoptysis → good pasture syndrome
• Kidneys
• Hematuria + hemoptysis + nasal crusting + nosebleeds → GPA
- Hematuria
Investigation
• +ve C-ANCA
PLABverse - plabverse@yahoo.com 6
 Rheumatology
Temporal arteritis (TA) or Giant cell arteritis (GCA)
➢ Vasculitis that affects the large arteries that supply the head, eyes and optic nerves
➢ New onset headache in any patient older than 50 years prompts consideration of this diagnosis, which if left
untreated may result in permanent vision loss
Features
• Headache → usually occurs in one or both temples
• Scalp tenderness, when comping hair
• Jaw claudication, pain when shewing
• Decreased/blurry vision, rarely sudden loss of vision
• Tongue numbness
+ Proximal stiffness (neck, arms, hips) due to polymyalgia rheumatica, a co-existing condition with TA
Investigation
• ESR → initial test
• Biopsy of the temporal arteries → Characteristic giant cells → confirmatory
Management
o Elevated ESR → Steroids (prednisolone) should be started immediately, before the biopsy is performed
o Add-on drugs
- Low-dose aspirin 75mg → reduces the rate of visual loss and strokes
- Bisphosphonates → reduces the risk of osteoporosis as a result of steroids especially in an elderly
female

PLABverse - plabverse@yahoo.com 7
 Rheumatology
Sjogren’s $
➢ Autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces
➢ May be seen alone or in association with other autoimmune diseases (secondary) such as rheumatoid
arthritis, primary biliary cirrhosis or SLE
➢ As it progresses, it becomes a systemic disease involving major organs (lungs, kidneys, etc.) and may
eventually evolve into a lymphoproliferative disease → malignant lymphoma
➢ Much more common in females (9:1)
Features
• Dry eyes (keratoconjunctivitis sicca)
- Itchy eyes, sandy feeling under their eyes (because of decreased lacrimal production)
• Dry mouth
- Patients may complain of dysphagia
- Can cause altered sense of taste
- Dry throat can lead to hoarseness of voice
• Vaginal dryness
• Bilateral parotid enlargement
Investigation
• Schirmer’s test → decreased tear production
• Rose Bengal stain → corneal ulceration
• ANAs → +ve, especially anti-Ro (SSA) and anti-La (SSB) [ROLA has Sjogren’s S]
• Rheumatoid factor → +ve
Management
• There’s no cure
• Artificial tears may help with the dry eyes (Hypromellose)
• Pilocarpine can also be used to stimulate the tear production

Mikulicz’s syndrome
➢ Persistent swelling of lacrimal and parotid (or submandibular) glands due to lymphocytic infiltration
➢ When no specific cause is found → Mikulicz’s disease, if 2ry to diseases like sarcoidosis or tuberculosis →
Mikulicz’s $
➢ If associated with sarcoidosis → bilateral perihilar lymphadenopathy may be present due to sarcoidosis
Features
1. Symmetrical enlargement of ALL salivary glands
2. Narrowing of the palpebral fissures due to enlargement if the lacrimal glands
3. Dryness of the mouth

- Both Mikulicz and Lofgren can be associated with sarcoidosis. However, Lofgren $ is characterized by erythema
nodosum, arthritis and bilateral hilar lymphadenopathy
- Sarcoidosis → non-caseating granulomas on biopsy
- TB → Caseating granulomas

PLABverse - plabverse@yahoo.com 8
 Rheumatology
Limited scleroderma vs Diffuse scleroderma

Limited scleroderma (CREST $) Diffuse scleroderma (systemic sclerosis)

Skin • Limited areas of skin are thick; usually • More areas are involved and thickened
just the fingers and/or face • Arms, legs and trunks are more affected
• Skin involvement doesn’t extend
above the elbow or above the knee
• NO trunk involvement

Onset • Slow • Rapid

Progression • Slow • Rapid

Length of skin • Raynaud’s phenomenon for many • Skin changes within 1 year of Raynaud’s
changes from years before any skin changes
Raynaud’s
phenomenon

Organ • Milder → less involvement • More severe → involvement of GIT, heart,

involvement • Lung involvement can be seen but lungs or kidneys
usually milder and less common

others CREST syndrome may be present • The skin can lose or gain pigment, making
areas of light or dark skin
• Calcinosis (Ca deposits)
• Raynaud’s phenomenon
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia (spider naevi)

Antibodies • Most have positive ANA • Most have positive ANA

• Antibodies to Scl-70 are usually • Anti-Scl-70 are usually positive (60%)
negative
Anti-Scl 70 is strongly associated with lung fibrosis
• Anti-centromere positive
and renal disease besides poor prognosis

Reynaud’s phenomenon
➢ Sudden episodes of pallor or cyanosis in response to cold or emotional stimuli
➢ Patients complain of cold sensitivity with other areas of skin affected (ears, nose and lower extremities)
➢ Attacks may begin in one or two fingers but typically involve all fingers and/or toes symmetrically and
bilaterally
➢ Could be secondary or associated with another disease (scleroderma)

- Vasoconstriction of blood vessels → Ischemia → pale

- Deoxygenation → blue
- After rewarming the hands, the blood flow will rebound (Reactive hyperemia) → red

PLABverse - plabverse@yahoo.com 9
 Rheumatology
De Quervain’s tenosynovitis
• Also called “Washerwoman sprain”, “Mummy thumb” or “Gamer’s Thumb”
• Caused by inflammation of the extensor pollicis previs and the abductor pollicis longus due to repetitive
stress injury
• Commonly occurs at in women following pregnancy due to the way in which the baby is lifted and held

PLABverse - plabverse@yahoo.com 10