Department of Internal Medicine, Maastricht University Medical Centre, Maastricht, the Netherlands.
Nephrol Dial Transplant. 2011 Dec;26(12):3960-7. doi: 10.1093/ndt/gfr149. Epub 2011 Mar 25.
Adult-onset Henoch-Schönlein purpura nephritis (HSPN) and primary IgA (IgAN) nephropathy have been considered indistinguishable immunohistopathologically and are often considered as two extremes of one disease entity. We postulate that adult-onset Henoch-Schönlein can be distinguished histologically from primary IgAN and that both diseases differ in their immunopathological mechanisms.
Twenty consecutive patients with adult-onset HSPN were studied. Serum was analysed for circulating IgA immune complexes; renal biopsies were analysed by light and electron microscopy (EM). As disease controls, 40 IgAN patients were studied.
Intracapillary leukocyte margination was seen in 15 of the 20 patients and cellular crescent formation in all renal biopsies of the HSPN patients. IgAN biopsies showed a few small crescents without intracapillary leukocytes. In 16 HSPN patients, EM was performed and in 10, no dense deposits were found. In all biopsies of IgAN patients, typical 'humps' were found. In 6 of 9 analysed HSPN patients, intermediate to large circulating immune complexes were found, whereas in 4 of 28 analysed patients with primary IgAN small circulating immune complexes were found.
We consider adult-onset HSPN distinguishable in histology and ultrastructure from primary IgAN. We believe adult-onset Henoch-Schönlein to be a circulating immune complex disease.
成人发病型过敏性紫癜肾炎（HSPN）和原发性 IgA（IgAN）肾病在组织病理学上难以区分，通常被认为是同一疾病实体的两个极端。我们假设成人发病型过敏性紫癜在组织病理学上可以与原发性 IgAN 区分开来，并且这两种疾病在免疫病理机制上存在差异。
连续研究了 20 例成人发病型 HSPN 患者。分析血清中的循环 IgA 免疫复合物；通过光镜和电子显微镜（EM）分析肾活检。作为疾病对照，研究了 40 例 IgAN 患者。
15 例 HSPN 患者的 20 例患者中存在毛细血管内白细胞边缘，所有 HSPN 患者的肾活检均存在细胞性新月体形成。IgAN 活检显示少量小新月体，无毛细血管内白细胞。在 16 例 HSPN 患者中进行了 EM，其中 10 例未发现致密沉积物。在所有 IgAN 患者的活检中均发现了典型的“驼峰”。在 9 例 HSPN 患者中分析了 6 例，发现了中等至大的循环免疫复合物，而在 28 例原发性 IgAN 患者中分析了 4 例，发现了小的循环免疫复合物。
我们认为成人发病型 HSPN 在组织学和超微结构上与原发性 IgAN 不同。我们认为成人发病型过敏性紫癜是一种循环免疫复合物疾病。
