于强－血管炎病诊断与治疗

1、 Vasculitides Fudan university Zhongshan Hospital Yuqiang 1 The term vasculitis refers to a heterogeneous group of disorders characterized pathologically by evidence of blood vessel inflammation and clinically by a diverse set of symptoms and signs.2Pathology three types： 1. necrotize 2. granulomas

2、3. only inflammatory cellular infiltration in the vessel wall 3Cause to 1.vessel wall angusty， blunting、 fibrosis， aneurysm 2. tissue ischemia， functional impaiement 4 CLASSIFICATION 1. histopathologic （1）necrotize （2）granulomas （3）leucocytoclasia （4）giant cell 52. clinical classific infectivity un-

3、infectivity63. clinical+ histopathologic primarily skin systemic secondary 7Lie CLASSIFICATION primarily large-vessel Takayasu artertis giant cell artertis Medium-vessel polyarteritis nodosa Churg-Struss syndrom Wegeners granulomatosis small-vessel Microscopic polyangiitis Schonlein-Henoch syndrom8S

4、econdary infectivity drug connective tissue disease associated with tumer9pathogenesis 1. infectivity 2. immune complex 3. antineutrophil cytoplasmic antibody ANCA-PR3 ANCA-MPO 4.antibody attack vessel AECA GBM antibody 5.cell immune 6.understand 10clinical manifestation fever debll general malaise、

5、 anaemia、 myosalgia、11Prompt: fever rash joint pain myosalgia organ impaired 12the Chapel Hill Consensus Conference 199313giant cell artertis: Granulomatous arteritis of the aorts and its major branches with a predilection for the extracranial branch of the carotid artery. Often involved the tempora

6、l artery .Usually occues in associated with polymyalgia rheumatica.Triad:1.temple pain 2.intermittence mandibular movement dyskinesia 3. acroiseageheadacheintermittence mandibular movement dyskinesiatemporal arteritisscalp painbiopsy 3/6 14Takayasu artertis Granulomatous inflammation of the aorta an

7、d its major branches.Usually occurs in patientsyounger then 5o.Agelimpingimpulse attenuateddifferential pressure20mmHgnoisearteriography 3/615polyarteritis nodosa Necrotizing inflammation of medium-size or small arteries without glomerulonephritis or vasculitis in arterioles, capilaries,or venules b

8、ody weight drawdown4kg livedo reticularis orchialgiamyosalgia NS. high BP Cr B hepatitisarteriography 3/1016Microscopic polyangiitis Necrotizing vasculitis , with few or no immune deposits ,affecting small vessel (capillaries , venules or arterioles) . Necrotizing arteritis involving small and mediu

9、m-size arteries may be present. Necrotizing glomerulnephritis is very common. Pulmonary capillaritis often occurs. Nephritis lung damage17Wegeners granulomatosis Granulomatous inflammation involving respiratory tract. And necrotizing vasculitis affecting small to medium-size vessels(capillaries, ven

10、ules,arterioles and arteries). Necrotizing glomerulonephritis is common.Triad: Upper airway lung nephritis18Churg-Struss syndrom Eosinophil-rich and granulomatous inflammation involving the respiratory tract. And necrotizing vasculitis affacting small to medium-size vessels, and associated with asth

11、ma and eosinophilia. Asthma Eosinophil-richneuritis lung infiltratenasosinusitis Eosinophil infiltrate 4/6 19BEHETS DISEASE it affects vessels of all sizes, leukocytoclastic vasculitides 1. aphthous stomatitis 2. genital aphthous ulcers, 3. uveitis, 4. cutaneous vasculitis, 5. pin-prick test 2/4 GI

12、disease, and meningoencephalitis. arthritis, 20Auxiliary examination 1. ANCA 2. AECA 3. vasography 4. biopsy 21DIAGNOSIS under study and the presence of pathognomonic clinical, laboratory, imaging, or histopathologic features. have been described in patients secondary vasculitis primary vasculitis systemic vasculitides.22differential diagnosis vi