《MPA》课件.pptVIP

好发于中老年男性，年龄40-50岁，男：女=1至1.8:1 好发于冬季，多有前驱感染的诱因。 全身症状：有发热、 乏力、 体重减轻、皮疹 肾脏病变最常见 ,蛋白尿、 镜下血尿、 管型尿 ,肾功能可进行性恶化。 呼吸系统：肺出血、 咯血、 呼吸困难、 肺间质病变 消化系统：消化道出血、 腹痛、 腹泻、 肝肿大 心血管系统：高血压、 心律失常、 心包炎、心肌梗死、 心力衰竭 其它：关节痛、 肌痛、 单或多发性神经病变、 巩膜炎 Alveolar septae with congestion, neutrophilic infiltrate, and fibrinoid necrosis of capillary walls. Adjacent alveolar spaces contain hemosiderin-laden macrophages, consistent with a history of pulmonary hemorrhage (hematoxylin and eosin, ?40).(Courtesy of Dr. C. Farver.) Glomerular filter consisting, from bottom to top, of fenestrated endothelium, basement membrane, and foot processes of epithelial cells. Note the filtration slits (arrows) and diaphragm situated between the foot processes. Note also that the basement membrane consists of a central lamina densa, sandwiched between two looser layers, the lamina rara interna and lamina rara externa.(Courtesy of Dr. Helmut Rennke, Brigham and Womens Hospital, Boston, MA.) * * Classification Diagrammatic representation of the typical vascular sites involved with the more common forms of vasculitis, as well as the presumptive etiologies. Note that there is a substantial overlap in distributions. Primary Vasculitides Predominantly Large Vessel Vasculitides Takayasu‘s arteritis Giant cell arteritis (temporal arteritis) Cogans syndrome Beh?ets disease Predominantly Medium Vessel Vasculitides Polyarteritis nodosa Cutaneous polyarteritis nodosa Buergers disease Kawasaki disease (MCLS) Primary angiitis of the central nervous system Predominantly Small Vessel Vasculitides Goodpastures disease (anti–glomerular basement membrane disease) Idiopathic Cutaneous Vasculitis （Cutaneous leukocytoclastic angiitis， hypersensitivity vasculitis) Henoch-Sch?nlein purpura Hypocomplementemic urticarial vasculitis Essential cryoglobulinemia Erythema elevatum diutinum ANCA-associated disorders Wegener‘s granulomatosis Microsc