Suppr超能文献

[肺出血肾炎综合征]

[Goodpasture disease].

作者信息

Fomegné G, Dratwa M, Wens R, Mesquita M, Van der Straaten M, Vanden Haute K, Fosso C

机构信息

Service de Néphrologie, Hôpital Erasme.

出版信息

Rev Med Brux. 2006 May-Jun;27(3):162-6.

Abstract

We report one case of acute renal failure with oliguria, microscopic haematuria and normocytic anemia in a 86-year old Swedish woman. A full investigation led to the diagnosis of Goodpasture disease, an isolated form of Goodpasture syndrome. Goodpasture disease is and autoimmune disorder characterized by the development of autoantibodies to the NC1 domain of the alpha3 chain of type IV collagen, found mainly in glomerular basement membranes (GBM). When the disease affects both the lung and the kidney, it is called Goodpasture syndrome but the pulmonary or renal involvement can be isolated or separated in years. Its pathogenesis is not well known. It occurs essentially in Caucasian subjects, preferentially from Nordic and Anglo-Saxon countries (higher prevalence of HLA DR B1-15 and B1-4 group). Are also mentioned, the exposure to hydrocarbons, rustproof, insecticides and greasy solvents. The annual incidence of Goodpasture syndrome is rare and has been estimated in Europe to be about 0.5 to 1 case per million inhabitants. The isolated renal form represents about 1/3 of the cases. The clinical presentation is characterized by rapidly progressive renal failure with oliguria or anuria and in case of lung involvement, pulmonary hemorrhage responsible of hemoptysis, sometimes massive. Renal biopsy and immunofluorescence analysis play a key role in the diagnosis. The presence of both linear deposits of IgG along the glomerular basement membrane (GBM) and circulating anti-GBM antibodies is of paramount importance. The treatment, which depends on the degree of renal involvement, is based on the association of corticosteroids, cyclophosphamide and plasma exchanges.

摘要

我们报告一例86岁瑞典女性急性肾衰竭病例,伴有少尿、镜下血尿和正细胞性贫血。全面检查后诊断为Goodpasture病,这是Goodpasture综合征的一种孤立形式。Goodpasture病是一种自身免疫性疾病,其特征是产生针对IV型胶原α3链NC1结构域的自身抗体,该结构域主要存在于肾小球基底膜(GBM)中。当该疾病同时累及肺和肾时,称为Goodpasture综合征,但肺或肾的受累情况可能是孤立的,也可能在数年后才出现。其发病机制尚不清楚。该病主要发生在白种人,北欧和盎格鲁-撒克逊国家的人群中更为多见(HLA DR B1-15和B1-4组的患病率较高)。还提到了接触碳氢化合物、防锈剂、杀虫剂和油脂溶剂等因素。Goodpasture综合征的年发病率很低,在欧洲估计约为每百万居民中有0.5至1例。孤立性肾型约占病例的1/3。临床表现为少尿或无尿的快速进行性肾衰竭,若累及肺部,则会出现咯血,有时咯血量大。肾活检和免疫荧光分析在诊断中起关键作用。沿肾小球基底膜(GBM)出现IgG线性沉积以及循环抗GBM抗体至关重要。治疗方法取决于肾脏受累程度,基于皮质类固醇、环磷酰胺和血浆置换联合应用。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验