Richman A V, Rifkin S I, McAllister C J
Hum Pathol. 1981 Jul;12(7):597-604. doi: 10.1016/s0046-8177(81)80042-1.
We present the clinical and pathologic data from two patients with rapidly progressive glomerulonephritis in whom the unusual combination of antiglomerular basement membrane antibody and immune complex disease was observed. In both patients the diagnosis of antiglomerular basement membrane disease was confirmed by renal tissue immunofluorescence and by positive assays for circulating antiglomerular basement membrane antibody. Ultrastructural studies revealed membranous nephropathy in one patient. Our data from this patient suggested evolution of pre-existing membranous nephropathy into antiglomerular basement membrane disease. In the second patient electron microscopy of renal tissue demonstrated numerous subendothelial, mesangial, and subepithelial deposits. It was impossible to ascertain in this patient whether antiglomerular basement membrane antibody or immune complex mediated injury was the primary pathogenetic event. Our data provide additional evidence for the rare clinical appearance of concurrent antiglomerular basement membrane and immune complex disease. Although the coexistence in both patients of antiglomerular basement membrane disease with immune complexes may have been coincidental, we think that this is unlikely. Rather our data suggest that the two mechanisms are causally related and that either one could have been the primary disease process.
我们展示了两名快速进展性肾小球肾炎患者的临床和病理数据,在这两名患者中观察到了抗肾小球基底膜抗体与免疫复合物疾病的不寻常组合。在两名患者中,抗肾小球基底膜疾病的诊断均通过肾组织免疫荧光和循环抗肾小球基底膜抗体检测呈阳性得以证实。超微结构研究显示其中一名患者存在膜性肾病。我们从该患者获得的数据提示,先前存在的膜性肾病演变为抗肾小球基底膜疾病。在第二名患者中,肾组织电子显微镜检查显示有大量内皮下、系膜和上皮下沉积物。在该患者中无法确定抗肾小球基底膜抗体或免疫复合物介导的损伤是否为主要致病事件。我们的数据为抗肾小球基底膜与免疫复合物疾病同时出现的罕见临床表现提供了更多证据。尽管两名患者中抗肾小球基底膜疾病与免疫复合物共存可能是巧合,但我们认为这种可能性不大。相反,我们的数据提示这两种机制存在因果关系,且其中任何一种都可能是原发性疾病过程。