Sjögren’s syndrome (SS) Sjögren’s syndrome (SS) n ition : Autoimmune inflammation of the exocrine (lacrimal, vary, etc) glands resulting in decreased production of tears va (xerophthalmia és xerostomia). ary and secondary forms (the latter accompanies other immun diseases (e.g.: RA, SLE, etc). emiol ogy : One of the most frequent autoimmune diseases; mated incidence: 1-10/1000, prevalence: 1%. Male: female o cca 1:9. ogene s is: Lymphoid infliltration of exocrine glands iltrating lymphocytes are mostly CD4+ T cells); with omitant inflammation and slow destruction.
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) . - PowerPoint PPT Presentation
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Sjögren’s syndrome (SS)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: femaleratio cca 1:9.
Pathogenesis: Lymphoid infliltration of exocrine glands(infiltrating lymphocytes are mostly CD4+ T cells); with concomitant inflammation and slow destruction.
Histology of salivary gland in Sjögren’s syndrome
Parotitisin SS
Keratoconjunctivitis sicca (rose bengal test) in SS
Schirmer’s test
Vasculitis (purpura)in Sjögren’s syndrome
Diagnostic criteria of SS Diagnostic criteria of SS (E(ECSGCSG, 1993), 1993)1. Ocular symptoms (at least one the following):
a/ symptomatic dry eyes for at least 3 monthsb/ repeated sensation of foreign bodies in the eyesc/ 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 monthsb/ recurrent salivary gland enlargementc/ requirement of frequent drinking while swallowing dry food
3. Positive Schirmer’s test ( 5mm/5 min) (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):
corticosteroid and/or Imuran or other immunosuppressivedrugs
3) parotitis: NSAID or corticosteroid (sialolithiasis or suppurative parotitis should be excluded!)
4) B cell lymphoma with moderate malignity
SclerodermaScleroderma
Definition: Inflammatory/degenerative disease of the connectivetissues with consecutive fibrosis. Skin, vessels, and muscles areinvolved, less commonly also internal organs (GI system, lungs, heart,kidney).Classification:1. Diffuse cutaneous scleroderma (progressive systemic
sclerosisnak; PSS)2. Limited cutaneous scleroderma (acrosclerosis forms, e.g. CREST)3. Overlap syndromes, mixed connective tissue disease (MCTD) and
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.
Raynaud’s phenomenon
Nailfold capillaries in scleroderma
Morphea (generalized)
Severe acrosclerosis
Calcinosis insclerodermaCalcinosis in scleroderma
Teleangiectasia in scleroderma
PSS
PSS
Centromere antibodies in acrosclerosis
Nucleolar antibodies in scleroderma
Bibasilar pulmonaryfibrosis in PSS
Classification criteria of sclerodermaClassification criteria of scleroderma (ARA, 1980)(ARA, 1980)
A/ Major criterium:1. Proximal scleroderma: symmetric thickening, tightening, and induration of 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 fingers3. 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 primary lung disease.
Definite diagnosis requires the major and 2 minor criteria.
SCLERODERMA-SCLERODERMA-LIKELIKE DISEASESDISEASES 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:
a) small amplitude, short polyphasic waves, b) fibrillation, irritability, c) spontaneous, bizarre discharges
4. Biopsy (=infiltration, necrosis, degenerative-regenerative signs5. Heliotrope rash*--------* Gottron’s papules or Gottron’s sign are thought to be more specificDiagnosis: PM = 4 criteria; DM = 5th criterium + 4 other)
Autoantibodies specific for PM/DM
a) anti- ‘synthetase’ antibodies, specific for ‘anti-synthetasesyndrome’anti-aminoacyl-tRNA synthetase antibodies:
b) anti- SRP (signal recognition particle), specific for anothersubgroup
c) other autoantibodies:anti-Mi-2 (antibody against a 220 kD nuclear protein)anti-MAS (antibody against a 4S sedimentation RNA)
Therapy of PM/DM:1) early diagnosis – early treatment!2) high dose corticosteroid (CS)3) in DM, especially when CS resistant: IVIG4) Imuran or methotrexate5) Other: e.g. cyclosporin
Relapsing Polychondritis
Definition: Rare destructing inflammatory disorder, frequently in relapsing form, affecting cartilages in many organs.
Pathogenesis: directly pathogenic autoantibodies against type II collagen and proteoglycan.