Habib R, Murcia I, Beaufils H, Niaudet P
INSERM U192, Hôpital Necker, Enfants Malades, Paris, France.
Biomed Pharmacother. 1990;44(3):159-62. doi: 10.1016/0753-3322(90)90003-r.
With the worldwide use of immunofluorescence microscopy, idiopathic IgA nephropathy (Berger's disease) has been recognized as a distinct form of primary glomerular disease. IgA nephropathy is the most common form of GN in many parts of the world. However, since the criteria for diagnosis are exclusively based on the findings of predominant IgA in the mesangium, the boundaries of this "entity" are not well delineated and it has become clear that several different clinical conditions share this common immunopathology. IgA nephropathy should, therefore be regarded as a syndrome. Schönlein-Henoch purpura (SHP) and Berger's disease represent the primary form of this syndrome. The clinical features of both diseases in children are reviewed. Although the clinical symptoms may appear different there is a close relationship between SHP and IgA nephropathy. They share the same pathology, the same pathogenesis, and they may both recur on a transplanted kidney. Moreover, both diseases can occur in a single family. When pathologic mechanisms are more defined, SHP may prove to be 1 end of a spectrum of diseases associated with microvascular immune deposits in which IgA is the predominant immunoglobulin seen, the other end being Berger's disease.
随着免疫荧光显微镜在全球范围内的应用，特发性IgA肾病（伯杰氏病）已被公认为一种独特的原发性肾小球疾病形式。IgA肾病是世界许多地区最常见的肾小球肾炎形式。然而，由于诊断标准完全基于系膜中主要为IgA的发现，这种“实体”的界限并未明确界定，而且很明显，几种不同的临床情况具有这种共同的免疫病理学特征。因此，IgA肾病应被视为一种综合征。过敏性紫癜（SHP）和伯杰氏病是该综合征的主要形式。本文回顾了儿童期这两种疾病的临床特征。虽然临床症状可能有所不同，但SHP与IgA肾病之间存在密切关系。它们具有相同的病理、相同的发病机制，并且都可能在移植肾中复发。此外，这两种疾病都可能发生在同一个家庭中。当病理机制得到更明确的定义时，SHP可能被证明是与微血管免疫沉积物相关的一系列疾病的一端，其中IgA是所见的主要免疫球蛋白，另一端是伯杰氏病。
