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Sjogren's syndrome
Sjogren's syndrome is an autoimmune disease that causes dryness in the body's moisture-producing glands due to antibodies attacking the glands. It most commonly affects the eyes and mouth, causing dryness, as well as other organs. Complications can include infections, kidney problems, or pancreatitis in rare cases. Symptoms include dry eyes and mouth, fatigue, joint pain, and lung involvement. The condition is diagnosed through tests of tear and saliva production, along with biopsies of salivary glands. Treatment focuses on replacing moisture and suppressing the immune system.
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Introduction to Sjögren's syndrome, its history, symptoms affecting moisture-producing glands.
Venus Williams discusses her Sjögren's syndrome diagnosis, symptoms, and misdiagnosis history.
Details on Sjögren's as a chronic autoimmune disease, forms, systemic manifestations, and prevalence.
Explains the immune response and factors in the development of Sjögren's syndrome, including inflammation.
Symptom overview including dry eyes, mouth, and effects on other body systems.
Lists additional non-glandular symptoms like arthritis, vasculitis, and systemic issues.
Investigation methods include blood tests, imaging, and tests to differentiate from other conditions.
Treatment options for symptoms of Sjögren's including artificial tears, medications, and lifestyle adjustments.
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Sjogren's syndrome
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- 2. Sjögren's syndrome wasin 1933 by Swedish eye specialist Henrik Sjögren • The condition causes antibodies to attack the body's moisture-producing glands, leading to dryness due to lack of secretions. • The illness most commonly affects the eyes, mouth, salivary glands, lungs, kidneys, skin and nervous system but all organs of the body can be affected. • In rare cases, complications from Sjogren's syndrome can cause salivary gland infections, kidney problems, ulcers or pancreatitis.
- 4. • Venus Williams,31, has spoken out about the autoimmune disease that caused her to drop out of the U.S. Open yesterday minutes before she was due to go on court. • Miss Williams was diagnosed with Sjogren's syndrome two weeks ago - after years of being misdiagnosed with other conditions. • 'For years I felt that I didn't have enough stamina and then, four years ago, I felt like I was not getting enough air but I was diagnosed with exercise-induced asthma,' she said. • 'The medicine for asthma never worked.' • Eventually, as her symptoms progressed doctors got to the bottom of the problem. The athlete now suffers from fatigue, swollen joints, dry eyes, dry mouth and heavy limbs. By Daily Mail Reporter UPDATED: 11:55 GMT, 2 September 2011
- 5. Sjögren's Syndrome • Achronic, slowly progressive autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes. • 1/3 have systemic manifestations • Few develop lymphoma • female-to-male ratio, 9:1
- 6. 2 Forms • PrimarySjögren's syndrome: The disease presents alone • Secondary Sjögren's : Asso. with other autoimmune diseases –RA –SLE –Scleroderma –Mixed CT disease –Primary biliary cirrhosis –Vasculitis –Chronic active hepatitis
- 7. Sjögren syndrome: Etiopathology •Etiology -not well understood • Findings suggest an ongoing interaction between the innate and acquired immune systems • Lymphocytic (T,B) infiltration of exocrine glands + • B lymphocyte hyper-reactivity
- 8. Sjögren syndrome: Etiopathology Glandularepith cells express MHC class II mols • Inherited susceptibility+ exo /endogenous antigens • Trigger a self-perpetuating inflammatory response • Continuing presence of active interferon pathways suggest ongoing activation of the innate immune system
- 9. Sjogren’s: Clinical Manifestation Themajority have symptoms related to diminished lacrimal and salivary gland function. In most, the primary syndrome runs a slow and benign course over 8 to 10 yrs.
- 10. Sjogren’s: Oral Manifestations •Dryness (xerostomia), burning sensation • Difficulty in swallowing dry food • Inability to speak continuously • Dental caries • Problems in wearing dentures Physical examination: • Dry, reddened, sticky mucosa • Atrophy of tongue • Saliva not expressible/ cloudy • Enlargement of salivary glands - 2/3 of primary SS - Uncommon in secondary
- 11. Dryness of themouth - xerostomia Deep red tongue Dental caries common
- 12. bilateral parotid enlargement-primary Sjögren syndrome.
- 13. Sjögren’s syndrome anda L parotid mass. Biopsy: B-cell lymphoma of mucosal lymphoid tissue
- 14. Sjogren’s: Ocular manifestations Dueto the destruction of corneal and bulbar conjunctival epithelium, defined as keratoconjunctivitis sicca • A sandy/ gritty feeling under eyelids • Burning • Accumulation of thick secretions at inner canthi • Decreased tearing, redness, itching, • eye fatigue, increased photosensitivity
- 15. Sjogren’s: Clinical Manifestations Otherexocrine glands • Resp : secretions dry nose, throat, and xerotrachea • GIT: Esophageal mucosal atrophy, atrophic gastritis, and subclinical pancreatitis • GUT: Dyspareunia and dry skin
- 16. Sjogren’s: Clinical Manifestation Medicationsthat cause similar symptoms • Antidepressants • Anticholinergics • Beta blockers • Diuretics • Antihistamines • Women on HRT • Anxiety
- 17. Sjogren’s: Clinical Manifestations ExtraGlandular: • Easy fatigability, low-grade fever, Raynaud‘s, myalgias, and arthralgias • Joints: Non-erosive arthritis • Pulmonary : Dry cough (small airway disease) • Renal: interstitial nephritis, acidosis and nephrocalcinosis. Glomerulonephritis rare (SLE overlaps). • Vasculitis -purpura, recurrent urticaria, skin ulcerations, GLN, and mononeuritis multiplex. • SN hearing loss, any focal deficits
- 18. Sjogren’s: Clinical Manifestations Glandular: •Xerophthalmia • Xerostomia • Bilateral parotid swelling Extraglandular: • Arthralgias/arthritis • Raynaud's phenomenon • Lymphadenopathy • Lung involvement • Vasculitis • Kidney involvement • Liver involvement • Lymphoma • Splenomegaly • Peripheral neuropathy • Myositis
- 19. Sjogren’s: Investigations Routine: • Mildnormochromic, normocytic anemia • ESR rise- in 70% Mouth: • Sialometry • sialography • Imaging: Ultrasound, MRI or MR sialography of salivary glands. • salivary gland biopsy- focal lymphocytic infiltrates.
- 20. Sjogren’s: Investigations Eyes: • Measurementof tear flow by schirmer‘s test • Tear composition • tear breakup time • tear lysozyme content • Slit-lamp examination after rose Bengal staining • punctuate corneal ulcerations • attached filaments of corneal epithelium
- 21. Schirmer test: Detectsdeficient tear production in Sjögren syndrome
- 22. Sjogren’s Investigations: Mustexclude • Hepatitis C virus infection should be ruled out since, apart from serologic tests, the clinicopathologic picture is almost identical to that of Sjögren's syndrome. • HIV infection • Sarcoidosis
- 23. Sjogren’s: Treatment • Artificialtears, Rx -corneal ulcerations • Avoid drugs that secretions (diuretics, anti HTs, anticholinergics & antidepressants) • Xerostomia: Best replacement - water • Vaginal dryness: Propionic acid gels • Secretagogues: Oral Pilocarpine / Cevimeline • Arthralgias : HCQ • RTA: Oral Soda bicarb • Systemic vasculitis: Steroids, immunosuppressives, M Abs • High-grade lymphomas: Chemo (CHOP) + M Abs v a g i n a l d r y n e s s