Group 3 Sjogrens Syndrome
Group 3 Sjogrens Syndrome
Group 3 Sjogrens Syndrome
CASE PRESENTATION dry eyes and dry mouth (sicca complex or primary
Sjögren’s syndrome), while others also develop
GUSTILO, NICOLE systemic lupus erythematosus, polyarteritis nodosa,
LACSON, COLEENE polymyositis or scleroderma, as well as rheumatoid
LERIO, FRANCINE arthritis (secondary Sjögren’s syndrome). As Sjögren
LIU, GRACE pointed out, cases of xerostomia and arthritis without
keratoconjunctivitis sicca have been observed.
I. CASE ❖ Sex: Women are much more likely to have Sjogren's
syndrome
A 48-year-old woman presents to the clinic with itching eyes, ❖ Rheumatic disease: It's common for people who have
dryness of mouth, difficulty swallowing, loss of sense of Sjogren's syndrome to also have a rheumatic disease
taste, hoarseness, fatigue and swollen parotid glands. She such as rheumatoid arthritis or lupus.
reports increasing joint pain over the past 2 years. She ❖ Ocular effects of Sjogren’s syndrome:
complains of frequent mouth sores. Laboratory tests reveal: - Failure of tear secretion
positive antinuclear antibodies and rheumatoid factor - Dried secretions stick to conjunctiva and cornea
levels of 70 U/ml. A parotid gland biopsy reveals inflammatory - Failure of clearance of foreign particles from the
infiltrates in the interlobular connective tissue with damage cornea and conjunctiva
to acinar cells and striated ducts. - Gritty sensation in the eyes
- Keratinisation and loss of goblet cells in
II. DIFFERENTIAL DIAGNOSIS conjunctiva
- Abrasions, ulcers and inflammation
- Risk of impairment or loss of sight
RULE IN RULE OUT
❖ Oral effects of Sjorgen’s syndrome:
- Discomfort
SYSTEMIC - DRY MOUTH - BUTTERFLY WING
LUPUS - DRY EYES LIKE RASH ON THE - Difficulties with eating or swallowing
ERYTHEMATOS - JOINT PAIN FACE - Disturbed taste sensation
US (SLE) - MOUTH SORE - Disturbed quality of speech
- Predisposition to infection
RHEUMATOID - DRY EYES - MORNING ❖ In radiographs:
ARTHRITIS - DRY MOUTH STIFFNESS Sialographs demonstrate the formation of punctate,
- JOINT PAIN - INFLAMMATION cavitary defects which are filled with radiopaque contrast
INVOLVING SMALL media. These filling defects have been said to produce a
JOINTS OF HANDS,
‘cherry blossom’ or ‘branchless fruit-laden tree’ effect
WRIST AND FEET
- JOINT radiographically.
DEFORMITIES
Revised international classification criteria for
SARCOIDOSIS - DRY EYES - NIGHT SWEATS sjogren’s syndrome:
- PAROTID - GRANULOMAS IN
GLAND SALIVARY GLANDS I. Ocular symptoms: a positive response to at least on of
ENLARGEMENT - COUGH UP BLOOD the three validated questions
- JOINT PAIN
1. have you had daily, persistent troublesome dry
eyes for more than 3 months?
2. Do you have a recurrent sensation of sand or
III. IMPRESSION (DIAGNOSIS) WITH gravel in the eyes?
SUPPORTING FACTS 3. Do you use tear substitutes more than three times
jogren's syndrome
S a day?
GROSS ANATOMY
Salivary glands are exocrine glands meaning that the Normal:
produced secretions are released via ducts into the
adjacent external or internal epithelia Parotid gland:
Submandibular gland:
Serous ● Dominant component in the
acini parotid gland and von Ebner
glands of the tongue
● Polygonal shaped cells with
dense, intracytoplasmic,
basophilic zymogen enzyme
containing granules
● Cells are periodic acid-Schiff
(PAS) reagent positive
Sublingual gland:
Mucous ● Dominant component in the
acini minor salivary glands of the
palate and base of tongue
● Round cells filled with mucin
and containing a basally
located, condensed nucleus
● Positive for mucicarmine,
alcian blue and PAS with
diastase resistance (DPAS)
Lacrimal gland:
Submandibular gland:
Sjögren’s
syndrome, minor salivary gland expression. Note
lymphocytic focus adjacent to intact acini.
Sublingual gland:
stochastic interactions between the environment and a
susceptible host culminating in a dysregulated immune
response and exocrine dysfunction.
Environmental Factors
PATHOPHYSIOLOGY
The two main symptoms of Sjogren's syndrome are:
The term Sjögren's syndrome refers to keratoconjunctivitis
sicca and xerostomia due to lymphocytic infiltrates of ● Dry eyes (keratoconjunctivitis sicca)
lachrymal and salivary glands. ● Dry mouth (xerostomia)
Role of Apoptosis
Complications:
> ↓ levels of C4
Immunofluorescent staining test denoting:
> ↑ CRP
This test measures systemic inflammation. It is elevated Ocular surface staining to look closely at the surfaces of
most commonly in the setting of an infection or tissue your eyes for damage and dryness.
injury, such as a myocardial infarction (heart attack). In the
setting of autoimmune disease, the CRP may be elevated,
but usually to a much lesser extent than during an
infection. 3. Imaging
> ↑ alkaline phosphatase, aspartate aminotransferase This special X-ray can detect dye that's injected into the
(AST), alanine aminotransferase (ALT) salivary glands in front of your ears. This procedure shows
how much saliva flows into your mouth.
There is a higher incidence of a type of liver disease known
as primary biliary cirrhosis in patients with Sjogren's - Salivary scintigraphy.
syndrome. A liver blood test (alkaline phosphatase) is
usually elevated in this disease.
This nuclear medicine test involves the injection into a vein
of a radioactive isotope, which is tracked over an hour to
● Complete Metabolic Panel: see how quickly it arrives in all your salivary glands.
The complete metabolic panel measures the electrolytes Your doctor might also do a lip biopsy to detect the
as well as chemicals or proteins in your blood that reflect presence of clusters of inflammatory cells, which can
the function of the kidneys, liver, and immune system. In indicate Sjogren's syndrome. For this test, a sliver of tissue
Sjögren’s syndrome, the total protein level may be is removed from salivary glands in your lip and examined
elevated, reflecting the increase in antibody (or under a microscope.
immunoglobulin) levels in your blood. The serum
bicarbonate or carbon dioxide level may be low, reflecting a
disorder of the kidney that may occur in Sjögren’s
VIII. MANAGEMENT OF THE DISEASE -
syndrome, known as renal tubular acidosis.
MEDICAL/SURGICAL/DENTAL
Treating Dry Eyes and Eye Inflammation Caused by
Sjogren’s Syndrome
● Artificial tears during day, gel at night
2. Eye tests
● Blepharitis – hot compress, eyelid cleansers,
eyelid massage
- Schirmer’s test ● Eye drops
● Punctal occlusions
Many people can treat their dry eyes with artificial tears
during the day and a gel at night. Some patients may have
eyelid inflammation (blepharitis) and benefit from the use of
hot compresses and eyelid cleansers, and gentle eyelid
massage to relieve blocked oil glands in the eyelids.
Others may require prescription eye drops.
For dry lips, petroleum jelly and lip balms can help.
Blocked nasal passages and a dry nose increase your
need to breathe through the mouth, which can make your
dry mouth worse. Saline nasal sprays can be helpful. Be
sure to treat other causes of congestion, such as allergies
and sinus infections, quickly. Dry skin usually improves
when you use skin lotion regularly and throughout the day,
especially after baths or showers. Some women
experience vaginal dryness, especially after menopause.