於强－血管炎病的诊断与治疗

null Vasculitides Fudan university Zhongshan Hospital Yuqiang 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. 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 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 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 CLASSIFICATION 1. histopathologic （1）necrotize （2）granulomas （3）leucocytoclasia （4）giant cell 2. clinical classific  infectivity  un-infectivity2. clinical classific  infectivity  un-infectivity3. clinical+ histopathologic primarily skin systemic secondary 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 syndromLie 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 syndromSecondary infectivity drug connective tissue disease associated with tumer 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 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、clinical manifestation fever debll general malaise、 anaemia、 myosalgia、Prompt: fever rash joint pain myosalgia organ impaired Prompt: fever rash joint pain myosalgia organ impaired the Chapel Hill Consensus Conference 1993the Chapel Hill Consensus Conference 1993giant 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. acroise age\headache\intermittence mandibular movement dyskinesia\temporal arteritis\scalp pain\biopsy 3/6 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. acroise age\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 patients younger then 5o. Age\limping\impulse attenuated\differential pressure>20mmHg\noise\arteriography 3/6Takayasu artertis Granulomatous inflammation of the aorta and its major branches.Usually occurs in patients younger then 5o. Age\limping\impulse attenuated\differential pressure>20mmHg\noise\arteriography 3/6polyarteritis 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 3/10polyarteritis 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 3/10Microscopic 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 damageMicroscopic 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 damageWegener’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 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 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 BEHÇET’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, BEHÇET’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 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.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 differential diagnosis viral infection malignancies Treatment 1. secondary vasculitis remove etiological factor 2. skin vasculitis antihistamine drug +NSAIDs 3. systemic vasculitides immune depressant Treatment 1. secondary vasculitis remove etiological factor 2. skin vasculitis antihistamine drug +NSAIDs 3. systemic vasculitides immune depressant prognosisprognosis