A Case of Sjogren's Syndrome

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A Case of Sjogren's Syndrome

  1. 1. Prof.S.SUNDAR’S unit Dr.G.Rengaraj.PG
  2. 2. History <ul><li>Mrs. Kalyani, a 60 yr female came with </li></ul><ul><li>c/o difficulty in swallowing – 2 years </li></ul><ul><li>- more for solid foods </li></ul><ul><li>- persistent </li></ul><ul><li>-not progressive </li></ul><ul><li>no h/o drooling of food or saliva </li></ul><ul><li>no h/o nasal regurgitation of food </li></ul><ul><li>no h/o hoarseness of voice </li></ul><ul><li>no h/o vomiting </li></ul><ul><li>h/o dryness of mouth – 2 yrs </li></ul>
  3. 3. History <ul><li>h/o dryness of both eyes – 2 yrs </li></ul><ul><li>- asso. With gritty sensation in both eyes - diff. in opening both eyes in morning </li></ul><ul><li>no h/o redness of eyes </li></ul><ul><li>h/o pain in both knee joint – 6 weeks </li></ul><ul><li>- more during activity, relieved by rest </li></ul><ul><li>- no diurnal variation </li></ul><ul><li>- no h/o any swelling in the joints </li></ul><ul><li>- no h/o any other joint involvement </li></ul>
  4. 4. History <ul><li>no h/o any chronic drug intake </li></ul><ul><li>Not a known DM/HT </li></ul><ul><li>No h/o any surgery or irradiation </li></ul><ul><li>No h/o jaundice </li></ul><ul><li>No positive family history </li></ul><ul><li>PM – attained menopause at 50 yrs of age </li></ul><ul><li>h/o cataract surgery done for both eyes 5 yrs before </li></ul>
  5. 5. Examination <ul><li>O/E pt. conscious, oriented,afebrile </li></ul><ul><li>no P/Ict./Cy./Cl./nodes/PE </li></ul><ul><li>dry tongue + </li></ul><ul><li>BP- 120/74 , P – 78/min </li></ul><ul><li>CVS- S1 S2 + , no murmurs </li></ul><ul><li>RS – NVBS + , no added sounds </li></ul><ul><li>P/A – soft , no organomegaly </li></ul><ul><li>CNS – NFND </li></ul><ul><li>Both eyes – Normal </li></ul>
  6. 7. DD of sicca symptoms <ul><li>Xerostomia </li></ul><ul><li>Viral infections </li></ul><ul><li>Drugs : 1.antihypertensive </li></ul><ul><li>2.parasympatholytic </li></ul><ul><li>DM </li></ul><ul><li>Trauma </li></ul><ul><li>Sjogren’s </li></ul><ul><li>Dry eye </li></ul><ul><li>Inflammation: </li></ul><ul><li>1.chronic conjunctivitis </li></ul><ul><li>2.chronic blepharitis </li></ul><ul><li>3. SJS </li></ul><ul><li>4. Pemphigoid </li></ul><ul><li>5. sjogren’s syn. </li></ul><ul><li>Toxicity </li></ul><ul><li>1. Burns </li></ul><ul><li>2. drugs </li></ul><ul><li>Misc. </li></ul><ul><li>1.trauma </li></ul><ul><li>2. hypovitaminosis A </li></ul>
  7. 9. INVESTIGATIONS <ul><li>Inv. Hb- 10.0 Sugar – 133 </li></ul><ul><li>PCV – 3O urea - 22 </li></ul><ul><li>RBC – 3 millions/ml creatinine – 0.8 </li></ul><ul><li>TC – 7000 Na – 138 </li></ul><ul><li>DC – P67L30 E3 k - 4.6 </li></ul><ul><li>ESR – 15 / 30 Bicarb - 22 </li></ul><ul><li>Plt. – 2 lakhs/ ml Cl – 98 </li></ul><ul><li>MCV - 92 </li></ul><ul><li>MCH- 32 </li></ul><ul><li>MCHC - 30 </li></ul>
  8. 10. Inv. <ul><li>Urine r/e alb – nil </li></ul><ul><li>sugar- nil </li></ul><ul><li>deposits- 1-2 pus cells/hpf </li></ul><ul><li>FBS – 78 , PPBS – 104 </li></ul><ul><li>CXR – Normal </li></ul><ul><li>ECG – Normal </li></ul><ul><li>X-ray both knee – normal </li></ul><ul><li>PS – Normocytic normochromic anemia + </li></ul>
  9. 11. Ophthal op. <ul><li>Rt. Eye Left eye </li></ul><ul><li>AS N N </li></ul><ul><li>Pupils irregular,sluggish RTL irregular, RTL </li></ul><ul><li>Lens pseudophakia pseudophakia </li></ul><ul><li>Refr. 6/24 5/60 </li></ul><ul><li>with PH no inc. in power 6/24 </li></ul><ul><li>SCHIRMERS TEST 2 mm 2 mm </li></ul><ul><li>imp - sugg. of DRY EYE </li></ul>
  10. 12. INV. <ul><li>RF – POS. </li></ul><ul><li>ANA - neg </li></ul><ul><li>Anti – SS-A - Positive </li></ul><ul><li>Anti – SS-B - Negative </li></ul>
  11. 13. Biopsy <ul><li>Lower lip biopsy: </li></ul><ul><li>Microscopic description: skin lined by hyperkeratotic,hyperplastic squamous epithelium with underlying dermis showing inflammatory cell infiltrate,congested blood vessels, basal layers showing basilar vacuolisation & edema of the dermis </li></ul><ul><li>Impression: consistent with sjogren’s syndrome </li></ul>
  12. 14. Inv. <ul><li>Anti-HCV – neg. </li></ul><ul><li>HIV – non-reactive </li></ul><ul><li>CT Chest – normal </li></ul><ul><li>ACLA – neg </li></ul>
  13. 16. Sjogren’s syndrome <ul><li>Revised International Classification Criteria for Sjögren's Syndrome </li></ul><ul><li>  </li></ul><ul><li>I. Ocular symptoms: a positive response to at least one of the following questions: </li></ul><ul><li>Have you had daily, persistent, troublesome dry eyes for more than 3 months? </li></ul><ul><li>Do you have a recurrent sensation of sand or gravel in the eyes? </li></ul><ul><li>Do you use tear substitutes more than 3 times a day? </li></ul><ul><li>  II. Oral symptoms: a positive response to at least one of the following questions: </li></ul><ul><li>Have you had a daily feeling of dry mouth for more than 3 months? </li></ul><ul><li>Have you had recurrently or persistently swollen salivary glands as an adult? </li></ul><ul><li>Do you frequently drink liquids to aid in swallowing dry food? </li></ul>
  14. 17. criteria <ul><li>III. Ocular signs-that is, objective evidence of ocular involvement defined as a positive result for at least one of the following two tests: </li></ul><ul><li>Schirmer's I test, performed without anaesthesia (</=5 mm in 5 minutes) </li></ul><ul><li>Rose bengal score or other ocular dye score (>/=4 according to van Bijsterveld's scoring system) </li></ul><ul><li>  IV. Histopathology: In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score >/=1, defined as a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm 2 of glandular tissue </li></ul>
  15. 18. CRITERIA <ul><li>V. Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests: </li></ul><ul><li>Unstimulated whole salivary flow (</=1.5 ml in 15 minutes) </li></ul><ul><li>Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts </li></ul><ul><li>Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer </li></ul><ul><li>  </li></ul><ul><li>VI. Autoantibodies: presence in the serum of the following autoantibodies: </li></ul><ul><li>Antibodies to Ro(SSA) or La(SSB) antigens, or both </li></ul>
  16. 19. Criteria <ul><li>Revised Rules for Classification </li></ul><ul><li>  </li></ul><ul><li>For primary SS </li></ul><ul><li>In patients without any potentially associated disease, primary SS may be defined as follows: </li></ul><ul><li>The presence of any 4 of the 6 items is indicative of primary SS, as long as either item IV (Histopathology) or VI (Serology) is positive </li></ul><ul><li>The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI) </li></ul><ul><li>The classification tree procedure represents a valid alternative method for classification, although it should be more properly used in clinical-epidemiological survey </li></ul><ul><li>  </li></ul><ul><li>For secondary SS </li></ul><ul><li>In patients with a potentially associated disease (for instance, another well defined connective tissue disease), the presence of item I or item II plus any 2 from among items III, IV, and V may be considered as indicative of secondary SS </li></ul><ul><li>  </li></ul><ul><li>Exclusion criteria: </li></ul><ul><li>Past head and neck radiation treatment </li></ul><ul><li>Hepatitis C infection </li></ul><ul><li>Acquired immunodeficiency disease (AIDS) </li></ul><ul><li>Pre-existing lymphoma </li></ul><ul><li>Sarcoidosis </li></ul><ul><li>Graft versus host disease </li></ul><ul><li>Use of anticholinergic drugs </li></ul>
  17. 20. Picture showing DRY,ERYTHEMATOUS ORAL MUCOSA of the pt <ul><li>The pt. had h/o dry mouth daily for 2 yrs & frequently drinking liquids to aid in swallowing solid food – satisfying item 2 of the criteria </li></ul>
  18. 22. Sjogren’s <ul><li>It is a chronic,slowly progressive autoimmune disease characterised by lymphocytic infiltration of the exocrine glands. </li></ul><ul><li>Middle-aged women are primarily affected, but it can occur in all ages,including childhood </li></ul><ul><li>F:M = 9:1 </li></ul><ul><li>Prevalence – 0.5 to 1.0 % </li></ul><ul><li>30 % of pts with autoimmune rheumatic disease suffers from secondary sjogrens syn . </li></ul><ul><li>Approx. 1/3 rd of pts present with systemic manifestations </li></ul>
  19. 23. Pathogenesis <ul><li>Autoimmune disease </li></ul><ul><li>Both T cell lymphocytic infiltration of exocrine glands & B cell lymphocytic hyperreactivity. </li></ul><ul><li>Infiltrating lymphocytes provide apoptotic signals to the epithelial cells. </li></ul><ul><li>The serum contains autoantibodies directed against non-organ-specific antigens such as immunoglobulins(RF) & extractable nuclear and cytoplasmic antigens ( Ro/SS-A, La/SS-B ) </li></ul><ul><li>HLA-B8, DR3 & DRw52 are prevalent in pts with primary sjogren’s. </li></ul>
  20. 24. Clinical manifestations <ul><li>Majority of pts have symptoms related to diminished lacrimal & salivary gland function </li></ul><ul><li>In most pts the primary syndrome runs a slow & benign course </li></ul><ul><li>Predominant Glandular manifestations: </li></ul><ul><li>xerostomia </li></ul><ul><li>keratoconjunctivitis sicca </li></ul><ul><li>other less frequent glandular manifestations: </li></ul><ul><li>xerotrachea,atrophic gastritis,subclinical pancreatitis </li></ul>
  21. 25. Clinical. <ul><li>Extra-glandular manifestations are seen in one-third of pts: </li></ul><ul><li>low-grade fever </li></ul><ul><li>raynaud’s phenomenon </li></ul><ul><li>myalgia </li></ul><ul><li>arthralgia </li></ul><ul><li>Most pts with primary sjogrens experience atleast one episode of nonerosive arthritis </li></ul><ul><li>renal involvement – interstitial nephritis clinically manifesting as hypoasthenuria </li></ul><ul><li>SN hearing loss was found in one-half of pts with sjogrens </li></ul><ul><li>Vasculitis – purpura, recurrent urticaria, skin ulcers </li></ul><ul><li>ILD </li></ul>
  22. 26. Clinical. <ul><li>A small but significant pts. Develop malignant lymphoma. The features sugg. The development of lymphoma are: </li></ul><ul><li>persistent parotid enlargement </li></ul><ul><li>purpura </li></ul><ul><li>leukopenia </li></ul><ul><li>cryoglobulinemia </li></ul><ul><li>Most lymphomas are extra-nodal marginal zone B cell lymphomas & are usually detected incidentally during evaluation of labial biopsy </li></ul>
  23. 27. Diagnosis <ul><li>The diagnosis of primary sjogren’s syndrome is obtained if </li></ul><ul><li>1)the patient presents with eye and/or mouth dryness, </li></ul><ul><li>2)the eye tests disclose keratoconjunctivitis sicca, </li></ul><ul><li>3)the mouth evaluation reveals classic manifestations of the syndrome, and </li></ul><ul><li>4) the pt serum reacts with Ro/SS-A and/or La/SS-B autoantigens </li></ul><ul><li>Labial biopsy is needed when the diagnosis is uncertain or to rule out other conditions </li></ul><ul><li>HCV infection should be ruled out since apart from SEROLOGIC TESTS the remainder of the clinico-pathologic picture is almost identical to that of sjogren’s </li></ul>
  24. 28. DD of sjogren’s HIV infection & sicca syndrome sjogren’s syn. sarcoidosis Age predominantly young males predominantly in middle-aged women invariable Auto-antibodies - Presence of autoantibodies to Ro/SS-A and/or La/SS-B - Histology lymphoid infiltrates of salivary glands by CD8+ lymphocytes Lymphoid infiltrates of salivary glands by CD4+ lymphocytes granulomas in salivary glands Genetics Associated with HLA-DR5 Associated with HLA-DR3 & DRw52 unknown Serology Positive tests for HIV Neg. tests Neg. tests
  25. 29. HIV & Sjogren’s <ul><li>Immunologic & rheumatologic disorders are common in HIV pts </li></ul><ul><li>Drug allergies are the most significant allergic reactions in HIV + , more common as the disease progresses(upto 65% for TMP/SMX) </li></ul><ul><li>SLE & RA are ameliorated by the concomitant HIV inf. </li></ul><ul><li>The one autoimmune disease that may occur with increased frequency in HIV inf. Is A VARIANT of PRIMARY SJOGREN’S </li></ul><ul><li>This syn. Consists of parotid gland enlargement,dry eyes & dry mouth </li></ul><ul><li>These pts donot have anti-SS-A or anti-SS-B </li></ul><ul><li>Less common with increased use of ARV </li></ul><ul><li>The term DIFFUSE INFILTRATIVE LYMPHOCYTOSIS SYNDROME(DILS) is used to describe this entity </li></ul>
  26. 30. Treatment <ul><li>Glandular manifestations: </li></ul><ul><li>Dry eyes:1.Artificial tears without preservatives </li></ul><ul><li>2.avoidance of drugs that decrease lacrimal & salivary secretion such as diuretics, antihypertensive drugs,anticholinergics & antidepressants. </li></ul><ul><li>3. systemic stimulation with oral pilocarpine 5 mg TID,oral cevimeline 30 mg TID </li></ul><ul><li>4. Severe dry eyes: NL duct occlusion ,soft contact lenses, corneal transplantation </li></ul>
  27. 31. Treatment <ul><li>Xerostomia: </li></ul><ul><li>Lubrication – water( the best replacement) </li></ul><ul><li>Local stimulation- flavoured lozenges or gum </li></ul><ul><li>Systemic stimulation – as for dry eyes </li></ul><ul><li>Topical application of fluoride </li></ul><ul><li>Oral candidiasis – topical nystatin or clotrimazole lozenges </li></ul><ul><li>Parotid enlargement : </li></ul><ul><li>Treat superinfection- antibiotics,analgesics </li></ul><ul><li>Hard, persistent – r/o lymphoma </li></ul>
  28. 32. Treatment <ul><li>Extraglandular manifestations: </li></ul><ul><li>Arthritis: Hydroxychloroquine 200-400 mg/day </li></ul><ul><li>Raynaud’s : cold protection with gloves & T.Nifedipine 10 mg TDS </li></ul><ul><li>RTA : Bicarbonate replacement </li></ul><ul><li>Lymphoma : CHOP + anti-CD20 </li></ul><ul><li>Systemic vasculitis: glucocorticoids 1 mg/kg per day </li></ul>
  29. 33. Secondary sjogren’s <ul><li>When it presents in association with other autoimmune rheumatic diseases </li></ul><ul><li>The common autoimmune diseases associated with sjogren’s are: </li></ul><ul><li>1. Rheumatoid arthritis </li></ul><ul><li>2. SLE </li></ul><ul><li>3. Scleroderma </li></ul><ul><li>4. MCTD </li></ul><ul><li>5. Primary biliary cirrhosis </li></ul><ul><li>6. vasculitis </li></ul><ul><li>7. chronic active hepatitis </li></ul>

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