Korzets Ze'ev, Magen Eva, Tetro Jane, Bernheim Joelle, Yaffe Daniel, Bernheim Jacques
Department of Nephrology, Meir Hospital, Kfar Saba, Israel.
Harefuah. 2002 Nov;141(11):960-3, 1010.
Henoch-Schonlein purpura (HSP) is a systemic vasculitic disorder involving both arterioles and capillaries. Although mainly a disease of early childhood, it can occur at any age. HSP is typically recognized as a syndrome with four major components: rash, joint manifestations, abdominal symptoms and renal disease. It is usually a mild condition with a tendency to relapses and generally has a good prognosis. Occasionally, however, it takes on an aggressive course. Gastrointestinal involvement is potentially the most serious complication of HSP. It may mimic an abdominal emergency and in its severest form result in small bowel infarction and/or perforation. Renal manifestations range from asymptomatic haematuria and/or proteinuria through a nephrotic syndrome to progressive glomerulonephritis leading to end stage renal failure. Apart from the major components outlined above, HSP may affect almost every other bodily organ. Vasculitis involving the myocard, lungs (pulmonary haemorrhage), ureter (stenosing ureteritis) and nervous system have been reported. We describe a case of HSP in a 50 year old woman which was complicated by the development of necrotizing crescentic glomerulonephritis and a left hemiparesis due to cerebral vasculitis. Interestingly, this patient had first appeared at the age of 9 years with a nephrotic syndrome and had been diagnosed by renal biopsy at the age of 31 as IgA nephropathy (IgAN). On her current admission, steroid and immunosuppressive therapy resulted in an improvement of renal function and an almost complete disappearance of her neurologic deficit.
过敏性紫癜(HSP)是一种累及小动脉和毛细血管的系统性血管炎疾病。虽然主要是儿童早期疾病,但可发生于任何年龄。HSP通常被认为是一种具有四个主要组成部分的综合征:皮疹、关节表现、腹部症状和肾脏疾病。它通常是一种轻度疾病,有复发倾向,总体预后良好。然而,偶尔也会呈侵袭性病程。胃肠道受累可能是HSP最严重的并发症。它可能类似腹部急症,最严重时可导致小肠梗死和/或穿孔。肾脏表现从无症状血尿和/或蛋白尿到肾病综合征,再到导致终末期肾衰竭的进行性肾小球肾炎不等。除上述主要组成部分外,HSP可能累及几乎所有其他身体器官。已有报道血管炎累及心肌、肺(肺出血)、输尿管(狭窄性输尿管炎)和神经系统。我们描述了一例50岁女性的HSP病例,该病例并发坏死性新月体性肾小球肾炎和因脑血管炎导致的左侧偏瘫。有趣的是,该患者9岁时首次出现肾病综合征,31岁时经肾活检诊断为IgA肾病(IgAN)。此次入院时,类固醇和免疫抑制治疗使肾功能得到改善,神经功能缺损几乎完全消失。