Sjogren syndrome by aseem

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Sjogren syndrome by aseem

  1. 1. SJÖGREN’S SYNDROME (SS)
  2. 2. INTRODUCTION • Syn : Gougerot–Houwer–Sjögren syndrome / Sicca Syn • Swedish Ophthalmologist Henrik Sjögren who first described it (1933) ; Mikulicz - 1888 • Defined as a systemic autoimmune disease caused by an immune-mediated inflammation of exocrine glands, and involves salivary, lacrimal and sweat glands, as Sicca Syndrome or with internal organ involvement.
  3. 3. CLINICAL TYPES • PRIMARY SS - de novo ; assoc with Malignant Lymphomas • SECONDARY SS - associated rheumatic disorder (RA / SLE / SSc / PBC) • SICCA SYNDROME – Xerophthalmia + Xerostomia – Internal Organ / Bone Inv
  4. 4. ETIOLOGY • Female : Male = 9 : 1 • 4/5/6th decade • Autoimmune ; HLA-B8 / DR3 / Complement allele C4AQO [7] / HLA-DRw52 (Jap) • Antibodies to the SSA / Ro in relatives
  5. 5. PATHOGENESIS • Lymphocyte and plasma cell infiltration  Autoantibody production (to ‘Ro’) • Connective tissue proliferation  Lymphocytic Activation • Glandular cell apoptosis  atrophy of glandular structures in affected tissues (salivary glands, sebaceous glands, sweat glands) • Secondary changes viz Conjunctival / Dacryoadenitis , Parotid Swelling , Angular Cheilits / Stomatitis
  6. 6. CLINICAL FEATURES CUTANOUS MANIFESTATIONS (50%) Xeroderma, pruritus and scaling Annular erythema, Papular Erythema including Sweet’s-like lesions (Jap) - graded Raynaud’s syndrome Vasculitis : Purpurae - Hyperglobulinemic Purpura and inflammatory vasculitis, including PAN-like lesions Vitiligo Sweating abnormalities Cutaneous Amyloidosis Alopecia—diffuse and generalized Pruritis Ani / Pruritis Vulva Nail fold Capillary Abnormalities (Splinter Hemorrhages / Fingertip Infarcts)
  7. 7. ANNULAR ERYTHEMA • A
  8. 8. SJOGREN VASCULITIS • A
  9. 9. XEROPHTHALMIA Aka DES Dry eyes for more than 3 months Gritty Sensation of sand or gravel in the eyes Need for tear substitutes more than 3 times a day Schirmer’s test, performed without anaesthesia (≤5 mm in 5 min) Rose Bengal score or other ocular dye score (Lisamine Green) Fluoroscine flow / Lactoferrin or Lysozyme estimation / Lacrimal Biopsy 43% - Keratoconjunctivitis Sicca with xerostomia 23% had associated CTD (RA) Oral symptoms may precede ocular, or both may occur late in the disease.
  10. 10. SCHIRMER’S TEST • German Ophthalmologist Otto Schirmer • determines whether the eye produces enough tears to keep it moist • This test is used when a person experiences very dry eyes or excessive watering of the eyes
  11. 11. PROCEDURE / INFERENCE • Schirmer's test places a small strip of filter paper inside the lower eyelids (conjunctival sac). The eyes are closed for 5 minutes. The paper is then removed and the amount of moisture is measured. This technique measures basic tear function. • A young person normally moistens 15 mm of each paper strip. Because hypolacrimation occurs with aging, 33% of normal elderly persons may wet only 10 mm in 5 minutes. Persons with Sjögren's syndrome moisten less than 5 mm in 5 minutes. • INTERPRETATION 1. Normal which is ≥15 mm wetting of the paper after 5 minutes 2. Mild which is 14-9 mm wetting of the paper after 5 minutes 3. Moderate which is 8-4 mm wetting of the paper after 5 minutes 4. Severe which is <4 mm wetting of the paper after 5 minutes.
  12. 12. • A
  13. 13. ROSE BENGAL DYE
  14. 14. CORNEAL STAINING SCORE (Van Bitzterveld Scoring System) NONE / MILD VARIABLE CENTRAL / DIFFUSE PUNCTATE
  15. 15. XEROSTOMIA • Saliva is at first thick and mucoid, but later salivary volume decreases; requirement of liquids to swallow food • Tongue is red, smooth and dry, and in severe cases there may be difficulty in swallowing dry food. • Parotid duct narrowing and web formation may develop. Recurrent episodes of swelling of one or both parotid glands or, less often, the submaxillary and sublingual glands, may be due to autoimmune inflammation or infection, which is common • Dental caries - severe and progressive • The lips are red, dry and scaly. There are frequently cracks at the corners of the mouth. • Chronic oral candidiasis is frequent
  16. 16. • AA
  17. 17. TESTING Unstimulated whole salivary flow (>1.5 ml in 15 min) Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary, or destructive pattern), without evidence of obstruction in the major ducts Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer Focal lymphocytic sialoadenitis (focus score ≥1) on HPE (the number of mononuclear cell infiltrates containing at least 50 inflammatory cells in a 4 mm2 glandular section)
  18. 18. UNSTIMULATED WHOLE SALIVARY FLOW • a
  19. 19. OTHER MANIFESTATIONS Arthralgia and arthritis Myalgia and myositis ENT : Sinusitis / Hearing Loss / TPRD / Atrophic Rhinitis GI : GERD / Achlorhydria Resp : Interstitial pneumonitis, pulmonary fibrosis and pulmonary hypertension Nephro : Interstitial nephritis, Renal Tubular Acidosis Neuro : migraine, neuropathies, cerebral vasculitis
  20. 20. MISCELLANEOUS • In patients without associated connective tissue disease, mild articular symptoms occur in 83%, with mild synovitis • Cervical or generalized LAN • Hepatosplenomegaly
  21. 21. • AA
  22. 22. American-EU Consensus Classification Criteria : SS (1989  1996) 1. Ocular symptoms: at least one of: 1 Dry eyes for more than 3 months 2 Gritty Sensation of sand or gravel in the eyes 3 Need for tear substitutes more than 3 times a day 2. Oral symptoms: at least one of: 1 Dry mouth for more than 3 months 2 Recurrently or persistently swollen salivary glands as an adult 3 Need liquids to swallow dry food 3. Ocular signs—at least one of the following two tests positive: 1 Schirmer’s test, performed without anaesthesia (≤5 mm in 5 min) 2 Rose Bengal score or other ocular dye score
  23. 23. 4. Histopathology: in minor salivary glands, focal lymphocytic sialoadenitis (focus score ≥1). 5. Salivary gland involvement: a positive result for at least one of the following diagnostic tests: 1 Unstimulated whole salivary flow (≤1.5 ml in 15 min) 2 Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary, or destructive pattern), without evidence of obstruction in the major ducts 3 Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer 6. Autoantibodies – SSA (Ro) / SSB (La)
  24. 24. Criteria For primary SS • In patients without any potentially associated disease, primary SS may be defined as follows: • a. The presence of any four of the six items is indicative of primary SS, as long as either item 4 (Histopathology) or 6 (Serology) is positive. • b. The presence of any three of the four objective criteria items (that is, items 3, 4, 5, 6) For secondary SS • In patients with a potentially associated disease, the presence of item 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS
  25. 25. Proposed classification criteria for SS : ACR (2012) • 1. Positive serum anti-SSA/Ro and/or anti-SSB/La or (positive rheumatoid factor and ANA titer 1:320) • 2. Labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score 1 focus/4 mm2 • 3. Keratoconjunctivitis sicca with ocular staining score 3 (assuming that individual is not currently using daily eye drops for glaucoma and has not had corneal surgery or cosmetic eyelid surgery in the last 5 years) Prior diagnosis of any of the following conditions would exclude participation in SS studies or therapeutic trials because of overlapping clinical features or interference with criteria tests: Head / Neck Radiation HCV Inf AIDS Sarcoidosis Amyloidosis GVHD
  26. 26. Disease Associations • • • • • • • • • • • • • • • Connective tissue disease (rheumatoid arthritis (26%), systemic sclerosis (22%) Sweet’s syndrome Lymphoproliferative disorders—B and T cell, and MALT associated Primary biliary cirrhosis Lipodystrophy Granulomatous panniculitis Behçet’s disease Coeliac disease Hypothyroidism and thyroiditis Myasthenia gravis Haemochromatosis Dermatitis herpetiformis Darier’s disease Sarcoidosis Waldenström’s hyperglobulinaemic purpura
  27. 27. DDx (SS) • HIV infection  diffuse infiltrative lymphocytosis syndrome (DILS), which is characterized by parotid enlargement; involvement of the renal, lung, and gastrointestinal systems • Chronic GVHD may mimic symptoms • SLE might be considered, especially at onset of the disease. Autoimmune thyroid dysfunction may be present.
  28. 28. DDx (SS) • • • • • • • • • • Amyloidosis Sarcoidosis Bulimia Pancreatitis, Chronic Polymyositis Rheumatoid Arthritis Salivary Gland Tumors, Major, Benign Salivary Gland Tumors, Minor, Benign Scleroderma Tuberculosis
  29. 29. DDx (SICCA SYN) • Medications (eg, antidepressants, anticholinergics, beta-blockers, diuretics, antihistamines, some antiarrhythmic and antiepileptic drugs) • Anxiety and depression • Complications from contact lenses • Dehydration / Age • Hypervitaminosis A • Mucous membrane pemphigoid • Environmental irritants • Mouth breathing • Chronic blepharitis • Chronic conjunctivitis • Rosacea • Therapeutic radiation or surgery to the head and neck • Parkinson disease • Amyloidosis • Sarcoidosis • Lymphoma
  30. 30. DDx (PAROTIDOMEGALY)
  31. 31. INVESTIGATIONS • Se Globulin • RA (52% Primary ; 98% Secondary) • ANA (>50%) speckled, and nucleolar factor is only occasionally found). • Anti-dsDNA / Anti-RNP rarely found in the sicca syndrome alone. • Anti-Ro (also called SS-A) and anti-La (SS-B) are frequently found (53%), associated with vasculitis, purpura, LAN • Antibodies to Lupus Anticoagulant / APLA • Antibodies to carbonic anhydrase 11 can be seen in patients with Sjögren syndrome who have primary billiary cirrhosis.
  32. 32. • Anti-La assoc with Annular Erythema • Antibody to salivary duct epithelium can be demonstrated in approximately 50% • Thyroglobulin antibodies are present in 25% of cases • Leukopenia and eosinophilia may be seen. ESR generally raised.
  33. 33. • Presence of anti–alpha-Fodrin antibody (reliable diagnostic marker of juvenile Sjögren syndrome) • Creatinine clearance may be diminished in up to 50% of patients • High alkaline phosphatase level – s/o Primary Biliary Cirrhosis • Elevated transaminase levels –s/o Chr Hepatitis • Hypokalemia
  34. 34. HPE • a
  35. 35. TREATMENT • Symptomatic treatment for the dryness of the eyes is best accomplished by lubricating agents, such as 0.5% Methylcellulose eye drops instilled into the eyes four or five times daily. • Bromhexine 16 mg three times daily has been found to increase the lacrimal secretion, but has no effect on salivary flow
  36. 36. • Artificial saliva / Steam inhalation / Humidifier – Respiratory Tract • Systemic corticosteroids reducing parotid swelling, but rarely increase parotid or lacrimal secretion. • Ciclosporin improved subjective xerostomia and may reduce histopathological progression. • Nifedipine may help Raynaud’s phenomenon. • Associated Polymyositis improved with monthly intravenous pulse CPA therapy. • Annular erythema in Japanese patients may be controlled by prednisolone 10–20 mg/day or by dapsone. • Graduated compression hosiery for hyperglobulinaemic purpura
  37. 37. SICCA SYN
  38. 38. BIOLOGICS • Reports on the use of Rituximab in patients with primary Sjögren syndrome - improved saliva flow rate, lacrimal gland function, vasculitis, and peripheral neuropathy • Eparazumab ?????
  39. 39. THANK YOU

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