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Sjögren’s syndrome (SS)

Sjögren’s syndrome (SS). Defin ition : Autoimmun e inflammation of the exocrine (lacrimal, salivary, etc) glands resulting in decreased production of tears and saliva (xerophthalmia és xerostomia) .

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Sjögren’s syndrome (SS)

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  1. Sjögren’s syndrome (SS) Definition: Autoimmune inflammation of the exocrine (lacrimal,salivary, etc) glands resulting in decreased production of tears and saliva (xerophthalmia és xerostomia). Primary and secondary forms (the latter accompanies otherautoimmun diseases (e.g.: RA, SLE, etc). Epidemiology:One of the most frequent autoimmune diseases; estimated incidence: 1-10/1000, prevalence: 1%. Male: female ratio cca 1:9. Pathogenesis:Lymphoid infliltration of exocrine glands (infiltrating lymphocytesare mostly CD4+ ab T cells); with concomitant inflammation and slow destruction.

  2. Histology of salivary gland in Sjögren’s syndrome Histology of salivary gland in Sjögren’s syndrome

  3. Parotitisin SS Parotitis in SS

  4. Keratoconjunctivitis sicca (rose bengal test) in SS Keratoconjunctivitis sicca (rose bengal test) in SS

  5. Schirmer’s test Schirmer’s test

  6. Vasculitis (purpura) in Sjögren’s syndrome Vasculitis (purpura) in Sjögren’s syndrome

  7. Diagnostic criteria of SS (ECSG, 1993) Diagnostic criteria of SS (ECSG, 1993) 1. Ocular symptoms (at least one the following): a/ symptomatic dry eyes for at least 3 months b/ repeated sensation of foreign bodies in the eyes c/ artificial tears are required at least 3 times a day 2. Mouth symptoms (at least one of the following): a/ symptomatic dry mouth for at least 3 months b/ recurrent salivary gland enlargement c/ requirement of frequent drinking while swallowing dry food 3. Positive Schirmer’s test ( 5mm/5min) (or positive rose bengal test) 4. Positive salivary gland (lower lip) biopsy. In histology at least 1 lymphoid follicle ( 50 cells) / 4 mm2 tissue) 5. Salivary gland involvement (at least one of the following): a/ positive scintigraphy b/ positive parotid sialography c/ unstimulated salivary flow ( 1.5 ml/15 min) 6. Autoantibodies (at least one of the following): a/ SS-A (Ro) or SS-B (La) antibody b/ antinuclear antibody (ANA) c/ rheumatoid factor (RF) Definite diagnosis requires at least 4 criteria. In the absence of other (systemic) autoimmune disease, the diagnosis is primary SS.

  8. Therapy of SS • Therapy of SS • symptomatic: artificial tears, physostigmine (?) • systemic manifestations: myositis, pneumonitis, neuritis – corticosteroid and/or Imuran or other immunosuppressivedrugs • parotitis: NSAID or corticosteroid (sialolithiasis or suppurative parotitis should be excluded!) • B cell lymphoma with moderate malignity

  9. Scleroderma • Definition:Inflammatory/degenerative disease of the connective • tissues with consecutive fibrosis. Skin, vessels, and muscles are • involved, less commonly also internal organs (GI system, lungs, heart, • kidney). • Classification: • Diffuse cutaneous scleroderma (progressive systemic sclerosisnak; PSS) • Limited cutaneous scleroderma (acrosclerosisforms, e.g. CREST) • Overlap syndromes, mixedconnective tissue disease (MCTD) and undifferentiatedconnective tissue disease (UCTD) • Localized scleroderma (morphea & linear scleroderma) • Epidemiology:not rare; incidence: 19/1 million; prevalence 19-75/100, • 000. More common in women;in the 30-55 ys age group, male: • female ratio is 7-12:1.

  10. Raynaud’s phenomenon Raynaud’s phenomenon

  11. Nailfold capillaries in scleroderma Nailfold capillaries in scleroderma

  12. Morphea (generalized) Morphea (generalized)

  13. Severe acrosclerosis Severe acrosclerosis

  14. Calcinosis in scleroderma Calcinosis in scleroderma

  15. Teleangiectasia in scleroderma Teleangiectasia in scleroderma

  16. PSS PSS

  17. PSS PSS

  18. Centromere antibodies in acrosclerosis Centromere antibodies in acrosclerosis

  19. Nucleolar antibodies in scleroderma Nucleolar antibodies in scleroderma

  20. Bibasilar pulmonary fibrosis in PSS Bibasilar pulmonary fibrosis in PSS

  21. Classification criteria of scleroderma (ARA, 1980) • Classification criteria of scleroderma(ARA, 1980) • A/ Major criterium: • Proximalscleroderma: symmetric thickening, tightening, and • indurationof the skin of the fingers and the skin proximal to the MCP • or MTP joints. The changes may affect the entire extremity, face, • neck, and trunk (thorax and abdomen). • B/ Minor kritériumok: • 2. Sclerodactyly: as above limited to the fingers • 3. Digital pitted scars or loss of substance from the fingerpad: depressed • areas at tips of fingers or loss of digital pad tissue as a result of ischemia. • 4.Bibasilar pulmonary fibrosis: bilateral reticular pattern of linear or • lineonodular densities most pronounced in basilar portions of the lungs • on standard chest roentgenogram: may assume appearance of diffuse • mottling or “honeycomb” lung. These changes should not be attributable • to primarylung disease. • Definite diagnosis requires the major and 2 minor criteria.

  22. SCLERODERMA-LIKE DISEASES SCLERODERMA-LIKEDISEASES EOSINOPHILIC FASCIITIS: (Shulman’s syndrome) diffuse fasciitis with eosinophilia) MIXED CONNESTIVE TISSUE DISEASE (MCTD ) (Sharp’s syndrome): A mixture of SLE, scleroderma, PM, RA (SS). Clinical picture: most prominent symptoms are: Raynaud’sphenomenon, synovitis: arthritis/arthralgia, „sausage-like" fingers, handsand/or sclerodactyly, esophagus motility disorder (dysphagia), myositis (CPK elevation), pneumonitis, pulmonary fibrosis. Laboratory: U1-RNP antibodies "OVERLAP" SYNDROMESAND UCTD UCTD = in most cases, preceding SLE or scleroderma

  23. Therapy of scleroderma • Therapy of scleroderma: • systemic: penicillamine (ineffective) • vasodilators (Ca-channel blockers, prostacyclin) • GI system: reflux - metoclopramide, slow motility – octreotid, antibiotics • pulmonary hypertension: ACE-inhibitors are ineffective, prostacyclin infusion • pneumonitis/fibrosis: corticosteroid/cytostatics • kidney: ACE-inhibitors

  24. Polymyositis/Dermatomyositis(Idiopathic inflammatory myopathies) Definition:a heterogenic group, characterized by proximal muscle weakness (involvement of shoulder and pelvic girdle). Classification: I. Adult polymyositis (PM) II. Adult dermatomyositis (DM) III. Myositis associated with malignancy IV. Childhood myositis V. Myositis associated with systemic autoimmune disease VI. Other (inclusion body, eosinophilic and localized)

  25. Gottron’s sign in DM Gottron’s sign in DM

  26. Gottron’s sign in PM Gottron’s sign in PM

  27. Splinter haemorrhage sign in PM/DM Splinter haemorrhage sign in PM/DM

  28. Heliotrope rash in DM Heliotrope rash in DM

  29. DM – shawl sign DM – shawl sign

  30. Lymphocytic infiltration in early PM Lymphocytic infiltration in early PM

  31. Classification criteria of PM/DM (Bohan & Peter, 1975) Classification criteria of PM/DM (Bohan & Peter, 1975) 1. symmetrical proximal muscular weakness 2. elevated serum enzymes (CPK, LDH, transaminases, aldolase) 3. Characteristic triad by EMG: a) small amplitude, short polyphasic waves, b) fibrillation, irritability, c) spontaneous, bizarre discharges 4. Biopsy (=infiltration, necrosis, degenerative-regenerative signs 5. Heliotrope rash* -------- * Gottron’s papules or Gottron’s sign are thought to be more specific Diagnosis:PM = 4criteria; DM =5th criterium + 4other)

  32. Autoantibodies specific for PM/DM Autoantibodies specific for PM/DM a) anti- ‘synthetase’ antibodies, specific for ‘anti-synthetase syndrome’ anti-aminoacyl-tRNA synthetase antibodies: anti-histidyl- (= Jo-1) anti-alanyl- (= PL-12) anti-threonyl- (= Pl-7) anti-isoleucyl-(= OJ) anti-glicyl- (= EJ) b) anti- SRP (signal recognition particle), specific for another subgroup c) other autoantibodies: anti-Mi-2 (antibody against a 220 kD nuclear protein) anti-MAS (antibody against a 4S sedimentation RNA)

  33. Therapy of PM/DM • Therapy of PM/DM: • early diagnosis – early treatment! • high dose corticosteroid (CS) • in DM, especially when CS resistant: IVIG • Imuran or methotrexate • Other: e.g. cyclosporin

  34. Relapsing Polychondritis Definition:Rare destructing inflammatory disorder, frequently in relapsing form, affecting cartilages in many organs. Pathogenesis:directly pathogenic autoantibodies againsttype II collagen and proteoglycan. Diagnostic criteria of polychondritis(McAdam et al, 1976) 1. 1. symmetrical auricular chondritis 2. 2. nonerosive, seronegative polyarthritis 3. 3. nasal chondritis 3. 4. eye involvement (conjunctivitis, keratitis, scleritis/episcleritis, uveitis) 5. 5. Upper airway (larynx-, trachea-) chondritis 6. 6. Cochlear and/or vestibular functional impairment (hearing loss, tinnitus, vertigo) Diagnosis: 3 (or more) criteria present. Therapy: CS, cyclosporine

  35. Polychondritis ofthe ear Polychondritis of the ear

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