Sjogren's syndrome

1. Sjogren's Syndrome

2. Sjögren's syndrome was in 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.

3. 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

4. Sjögren's Syndrome A chronic, 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

5. 2 Forms Primary Sjögren'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

6. 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

7. Sjögren syndrome: Etiopathology Glandular epith 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

8. Sjogren’s : Clinical Manifestation The majority 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.

9. 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

10. Dryness of the mouth - xerostomia Deep red tongue Dental caries common

11. bilateral parotid enlargement- primary Sjögren syndrome.

12. Sjögren’s syndrome and a L parotid mass. Biopsy: B-cell lymphoma of mucosal lymphoid tissue

13. Sjogren’s : Ocular manifestations Due to 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

14. Sjogren’s : Clinical Manifestations Other exocrine glands Resp : secretions dry nose, throat, and xerotrachea GIT: Esophageal mucosal atrophy, atrophic gastritis, and subclinical pancreatitis GUT: Dyspareunia and dry skin

15. Sjogren’s : Clinical Manifestation Medications that cause similar symptoms Antidepressants Anticholinergics Beta blockers Diuretics Antihistamines Women on HRT Anxiety

16. Sjogren’s : Clinical Manifestations Extra Glandular: 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

17. 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

18. Sjogren’s : Investigations Routine: Mild normochromic , normocytic anemia ESR rise- in 70% Mouth: Sialometry sialography Imaging: Ultrasound, MRI or MR sialography of salivary glands. salivary gland biopsy- focal lymphocytic infiltrates.

19. Sjogren’s : Investigations Eyes: Measurement of 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

20. Schirmer test : Detects deficient tear production in Sjögren syndrome

21. Sjogren’s Investigations : Must exclude 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

22. Sjogren’s : Treatment Artificial tears, 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 vaginal dryness