Vasculitides  Fudan university  Zhongshan Hospital Yuqiang 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. Pathology  three types ：1. necrotize 2. granulomas 3. only inflammatory cellular   infiltration in the vessel wall  Cause to 1.vessel wall  angusty ， blunting 、 fibrosis ， aneurysm   2. tissue  ischemia ，  functional impaiement CLASSIFICATION   1. histopathologic  （1 ）necrotize （2 ）granulomas （3 ）leucocytoclasia  （4 ）giant cell   2. clinical classific infectivity un-infectivity 3. clinical+ histopathologic primarily skin  systemic secondary Lie CLASSIFICATION primarily large-vessel  Takayasu artertis giant cell artertis Medium-vessel polyarteritis  nodosa  Churg-Struss syndrom  Wegener’s granulomatosis  small-vessel  Microscopic polyangiitis    Schonlein-Henoch syndrom Secondary infectivity  drug  connective tissue disease  associated with tumer  pathogenesis  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 clinical manifestation   fever  debll   general malaise 、anaemia 、myosalgia 、Prompt: fever  rash  joint pain  myosalgia  organ impaired  the Chapel Hill Consensus Conference 1993  giant cell artertis: Granulomatous arteritis of the aorts and it’s major branches with a predilection for the extracranial branch of the carotid artery. Often involved the temporal artery .Usually occues in associated with polymyalgia rheumatica.Triad:1.temple pain 2.intermittence mandibular movement dyskinesia 3. acroiseage\headache\intermittence mandibular movement dyskinesia\temporal arteritis\scalp pain\biopsy 3/6  Takayasu artertis  Granulomatous inflammation of the aorta and its major branches.Usually occurs in patientsyounger then 5o.Age\limping\impulse attenuated\differential pressure>20mmHg\noise\arteriography 3/6  polyarteritis  nodosa  Necrotizing inflammation of medium-size or small arteries without glomerulonephritis or vasculitis in arterioles, capilaries,or venules body weight drawdown>4kg \livedo reticularis orchialgia\myosalgia\ NS. \ high BP \ Cr \ B hepatitis\arteriography 3/10  Microscopic polyangiitis Necrotizing   vasculitis , with few or no immune deposits ,affecting small vessel (capillaries , venules or arterioles) . Necrotizing arteritis involving small and medium-size arteries may be present. Necrotizing glomerulnephritis is very common. Pulmonary capillaritis often occurs. Nephritis lung damage Wegener’s granulomatosis  Granulomatous inflammation involving respiratory tract. And necrotizing vasculitis affecting small to medium-size vessels(capillaries, venules,arterioles and arteries). Necrotizing glomerulonephritis is common.Triad: Upper airway lung nephritis  Churg-Struss syndrom  Eosinophil-rich and granulomatous inflammation involving the respiratory tract. And necrotizing vasculitis affacting small to medium-size vessels, and associated with asthma and eosinophilia.  Asthma\ Eosinophil-rich\neuritis\ lung infiltrate\nasosinusitis\ Eosinophil infiltrate 4/6 BEH ET’S 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 disease, and meningoencephalitis. arthritis,   Auxiliary examination 1. ANCA 2. AECA  3. vasography  4. biopsy DIAGNOSIS  under study and the presence of pathognomonic clinical, laboratory, imaging, or histopathologic features. have been described in patients  secondary vasculitis  primary vasculitis   systemic vasculitides.  differential diagnosis   viral infection malignancies Treatment  1. secondary vasculitis  remove  etiological factor  2. skin vasculitis antihistamine drug +NSAIDs 3. systemic vasculitides immune depressant prognosis *  *