Autoimmune diseases nLupus erythematosus nScleroderma nDermatomyositis nOverlap syndromes ( e.g. Sharp syndrome) nVasculitis Lupus erythematosus nsystemic lupus erythematosus - SLE n nsubacute cutaneous LE – SCLE n nchronic cutaneous LE – CCLE n /chronic discoid LE – CDE/ n nLE in newborns nMedicine induced ( hydralazine, sulfonamides) n n Systemic lupus erythematosus - SLE nARA criteria: nButterfly rash nDiscoid lesions nphotosensitivity nOral lesions narthritis nserositis- pericarditis, pleuritis… nneurologic diseases nKidney involvement ( proteinuria 0,5g/d) nHematological abnormalities (leukopenia) nImmunological abnormalities ( LE cells, n ANA homogenous, periferal, dsDNA, Sm) ANAhom sle-cde-oblicej sle-motylek-muz Chronic cutaneous LE - CCLE (CDE) nPhotosensitivity nDiscoid lesions - CDE nHypertrophic lesions - lupus tumidus nLupus paniculitis nANA only low titers, granular type nNot systemic disease n nSymptoms of SLE 122-2298_IMG cde-oblicej cde-oblicej-zena cde-oblicej-muz Subacute cutaneous lupus erythematosus - SCLE nAnnular lesions or papulosquamous lesions nphotosensitivity nANA, anti Ro/ SSA, La/SSB granular type nMild systemic disease (neurologic symptoms, myalgia, arthralgia, rarely nefritis) nneonatal LE: newborns – mother with + anti SSA: cutaneous symptoms type SCLE a complete AV block, necessity of pacemaker! scle-trup-celek-zena 110-1079_IMG xxx xxx Dg: histopathology, direct immunofluorescence (lupus band) Lupus erythematosus nUVA, UVB protection !!! nantimalarials (chloroquine, hydroxychloroquine) n lower absorption of UV radiation n stabilization of lysosomal membranes (antiinflammatory effect) n mild immunosuppressive effect nAdverse effects: n retinopathy n GIT problems, bone marrow depression, hepatotoxicity, rashes n n Therapy: ncorticosteroids nimmunosuppressants (cyclophosphamide) nNSAID (DMARDs) nthalidomide nplasmaferesis, puls therapy atd. n Systemic sclerosis nDisturbance of synthesis of collagen n autoimmune disease: T ly ->IL-2,3,6 -> n fibroblasts -> overproduction of collagen V -> fibrosis nImmunologic abnormalities – n antibodies (anti Scl70, ANA , anti -centromeras) n defect of immune cells nVessel changes – involvement of endothelium Plné kosočtverce SS – types: nLimited (acrosclerosis, sclerodactyly) n Raynaud‘ s phenomenon n hard skin of fingers, sclerodactyly n ulcerations on distal digits n hypomimia, microstomia nDiffuse nCREST syndrome sclerodermia-diffusa-sklerodatylie sclerodermia-diffusa-obl sklerodermie-difusni-oblicej-zena-u akrocyanoza-ss Systemic sclerosis nARA criteria: nproximal scleroderma nBilateral lung intersticial fibrosis nLoss of tissue in distal digits (fingertips) nSclerodactyly, thickening and swelling of the fingertips nOther signs: nRaynaud‘s phenomenon nEsophageal changes nRenal involvement - nephrosclerosis nPulmonary hypertensis npericardial effusion, myocardial fibrosis CREST syndrome nC alcinosis nR aynaud‘s phenomenon nE sophageal changes nS cleroderma nT eleangiectasias nanti centromere antibody Scleroderma nTherapy: n corticosteroids n immunosuppressants /Aza, CF, MTX/ nd-penicilamine n - lower synthesis of collagen n - but: frequent adverse effects! nPUVA, UVA-1, bath PUVA nIFN g - lower synthesis of collagen nvasoactive and rheologic medication (pentoxiphylline, prostaglandins) ncalcium antagonists – nifedipin n nprokinetics, antacids nphysical therapy Localised scleroderma nMorphea /isolated patches of hardened skin/ nguttate nlinear ngeneralised (pansclerotic morphea) nsubcutaneous – eosinophilic fasciitis nANA – only low titers nNo systemic involvement morfea-bok-zena morfea-detail-lilac-ring morphea-kriz sklerodermie-linearni-stehno Localised scleroderma nTherapy: nLocal CS, masage with Heparoid ung nAnalogs of vit. D3 - calcipotriol nLocal PUVA nP-PNC, penicilamine nAntimalarials nPUVA nretinoids Dermatomyositis nJuvenile type – association with infections nAdult type - association with tumours (paraneoplasia) nheliotropic rash, Gottron‘s sign, poikilodermatitis, erytematous lesions nEMG nANA, anti Jo-1 / histidyl –t-RNA synthetase/ PM>DM n anti Mi-2 / nucleoproteins 30-240 kDa/ DM nCK, LDH, GGT, ALD, AST, ALT, myoglobin nHistopathology – muscle, skin dmkrut-celo dmkrut-hrud dmkrut-ruce dmkrut-prsty 118-1847_IMG 118-1821_IMG Dermatomyositis ncriteria ARA: 1) symetric proximal muscle weakness n 2) elevated levels of CK, LD or myoglobin n 3) emg signs of myopathy n 4) muscle biopsy with features of inflammatory myopathy n 5) typical skin lesions of DM n ndefinite dg DM : at least 3 from the first, 4 + 5 nsupposed: at least 2 from the first, 4 + 5 nautoAb: ANA posit in 50%, n others:: anti Mi-2 /nucleoproteins 30-240 kDa / 10-30% n anti Jo-1 / histidyl-tRNA syntetase/ 15-40% n n Dermatomyositis - therapy nCorticosteroids - inicial and long term dose nImmunosuppressants - MTX, azathioprine, cyclosporine nAntimalarials – only in skin involvement n nIVIG nrituximab Overlap syndromes nMCTD – Sharp´s syndrom n S Scl, PM/DM sometimes RA nScleromyositis SScl/PM nSScl/SLE n nSScl/RA nSLE/PM