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Vasculitides

Vasculitides. Classification, patogenesis, diagnostics and therapy Ústav imunologie UK 2.LF, Praha. Systémové vaskulitidy - etiologie. Etiology: primary vasculitides secondary vasculitides Causes of secondary vasculitides : External antigens (infections, allergy-drugs, serum sickness)

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Vasculitides

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  1. Vasculitides Classification, patogenesis, diagnostics and therapy Ústav imunologie UK 2.LF, Praha

  2. Systémové vaskulitidy - etiologie Etiology: • primary vasculitides • secondary vasculitides Causes of secondary vasculitides: • External antigens (infections, allergy-drugs, serum sickness) • Internal antigens (autoimmune diseases, tumors) • Postirradiation vasculitis, GVHD

  3. Classification of primary vasculitides • Vessels size (small, medium, large) • Histological classification according to the infiltration (leucocytoklastic, lymfocytic) • Patogenetic classification (later on) • Serologiccal class. (ANCA+,ANCA-) • nosologic units Chapell-Hill Consensus on Nomenclature of Vasculitis, 1992

  4. Patogenetic classification II.Type III.type, mostly Secondary vasculitides Stimulation of target Cells – functional consequences

  5. ANTIBODY MEDIATED GLOMERULONEPHRITIS Circulating anti-GBM antibodies with linear glomerular IF staining Glomerular immune complex localization with granular IF staining Little or no glomerular IF immunoglobulin staining Anti-GBM glomerulonephritis Idiopathic (pauci-immune) glomerulonephritis Immune complex glomerulonephritis Ch.Jennette, 2002

  6. ANCA - antineutrophil cytoplasmic autoantibodies target antigens – granules of PMN proteinase 3 (PR3) myeloperoxidase (MPO) Bactericidal and permeability increasing protein (BPI) lactoferin, elastase,... C-, P-, A-ANCA

  7. Diagnostic association of ANCA C-ANCA Dg / target Ag Wegener gr. / PR3 Cystic fibrosis / BPI P-ANCA Dg / target Ag Microscopic polyangiitis, iRPGN / MPO RA, JCA/ MPO, LF C,P,A-ANCA Dg / cílový antigen UC / different Autoim.hepatitis/ different ANCA POSITIVITY DO NOT MEANS AUTOMATICALLY DG OF VASCULITIS

  8. Wegener granulomatosis sinusitis Lung infiltration glomerulonefritis C-ANCA, PR3-ANCA Microscopic polyangiitis - MPA systemic (lung, kidneys..) Limited to kidneys – idiopatic RPGN P-ANCA, MPO-ANCA Characteristic of vasculitic syndromes

  9. Wegener granulomatosis, men, 23 y

  10. RPGN - „crescents“ (extracapillary proliferation)

  11. Kawasaki disease children Fever, conjunctivitis, lymph node enlargement, scarlet exantema 15% vasculitis of coronary vessels AEAb, ANCA, Vbeta 2,8 - superantigens Goodpasture sy pulmorenal sy anti-GBM nefritis anti-GBM Ab (colagen IV) 3-15% ANCA Characteristic of vasculitic syndromes - 2

  12. Churg-Strauss sy (Allergic granulomatous angiitis, AGA) astma Lung infiltration capilaritis of lung vessels eosinofilia >1500 P-ANCA, MPO cca 50% Polyarteriitis nodosa Muscular arteries medium size kidney, nerves, muscules, skin (P-ANCA) Charakteristic of vasculitic syndromes - 3

  13. AGA - Churg-Strauss sy, before and after therapy

  14. Polyarteriitis nodosa - Strictures of muscular arteries medium sized

  15. Characteristic of vasculitic syndromes - 4 Henoch-Schonlein purpura • purpura • hemorhagic enteritis • artritis • nefritis • Immune complex with IgA (similar to IgA nephropathy – limited form of H-S) • ANCA < 10%

  16. Takayasu arteritis Younger patients females:males 7:1 Aortal arch, renal arteries Malignant hypertention ANCA negative Horton temporal arteritis Older patients 3:2 polymyalgia rheumatica Carotis and its branches amaurosis ANCA negativní Characteristic of vasculitic syndromes - 5

  17. Takayasu and Horton arteritis

  18. Behcet disease Oral and genital ulceration iridocyklitis,uveitis Skin necrosis CNS involvement HLA B5 Buergerova choroba (trombangitis obliterans) smokers Tromboflebitis migrans Vessels occlusions inflammation It is not a vasculitis, but primary trombosis Characteristic of vasculitic syndromes - 6

  19. Patients history and clinical symptoms Laboratory findings Imaging methods histology General symptoms, organ symptoms non/specific rtg angiography,NMR Histology classical imunohistochemistry Diagnosis of vasculitides - 1

  20. Diagnosis of vasculitides -2 • General symptoms: fever, weight loss, fatigue, myalgia, arthralgia • lungs: hemoptysis, cough, dyspnea, astma, infiltration • ORL: sinusitis, otitis, hearing loss • skin: purpura, pyodermie, necrosis, EN • ocular: uveitis, iridocyclitis,episcleritis

  21. Diagnosis of vasculitides - 3 • CNS and peripheral nerves: polyneuropathy, paresis, epileptic paroxysm, psychosis • kidney: hypertension, proteinuria, erytrocyturia, renal failure

  22. INDUCTION corticosteroids - pulses, classical Imunosupressives (CFA) plasmapheresis ivig MoAb MAINTENANCE corticosteroids (classical, alternative) immunosupressives (azathioprin, MTX) TMP/SMX Therapy of vasculitides studies EUVAS (MEPEX, CYCAZAREM)

  23. Prognosis of vasculitides • Prim. vasculitides are serious illness with uncertain prognosis – early complications (renal failure, lung hemorhagia), late – atherosclerosis, immune deficiency do to immunosuppresion • Prognosis is dependent on early diagnostic • Early laboratory diagnostic possible in AAV, but not every ANCA+ is vasculitis

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