抗肾小球基膜抗体疾病的临床及病理分析

Clinical and pathological aspects of anti-glomerularbasement membrane disease

７例抗肾小球基膜抗体疾病占全部肾活检病例的０．１４％，男女比为６∶１，平均起病年龄２６．１±７．６岁。其临床表现为前驱性呼吸道病毒感好（５／７），肺出血（５／７），肾脏受累（７／７），高血压（７／７），中重度贫血（７／７）及中枢神经系统受累（１／７）。肾脏受累表现为镜下血尿或肉眼血尿（７／７）、中度蛋白尿（６／７）及急进性肾功能减退（７／７）；肾脏病理改变的特征为新月体形成及线状ＩｇＧ沉积物沿毛细血管袢基膜弥漫沉积，伴突出的间质改变。重复肾活检早期有效的治疗可显著减少新月体形成的肾小球比例及细胞性新月体比例，同时细胞性新月体可在短期内向纤维细胞性新月体或纤维素性新月体转化，使肾小球发生球性硬化。早期血浆置换及甲基强的松龙冲击治疗可缓解肺、肾损害，而缓解后停用免疫抑制治疗则可能导致复发。

nti-glomerular basement membrane ( GBM )disease is a rare disease which is mediated by theautoantibodies to GBM. In this study, we retro-spectively analyzed the clinical and pathological dataof 7 cases of anti-GBM disease, the diagnosis ofwhich were comfirmed by renal biopsy. Anti-GBMdisease accounted for 0. 14% of patients who under-went renal biopsy. The cardinal clinical manifesta-tion of Anti-GBM disease includes : predromatic up-per respiratory viral infection ( 5/7 ) , alveolar hemor-rhage, rapidly progressive renal failure, anemia andCNS involvement. The characteristic change in re-nal biopsy is the linear IgG immunodeposits alongthe GBM and the crescentic glomerulonephritis.The tubulointerstitial changes are remarkable withprominent infiltration of monocytes and lympho-cytes. Monoclonical antibody studies indicated thatCD+ was the dominant subtype of the infiltrating Tcells both in the glomerular and in the interstitiumarea , as compared with CD+ . The study shows ear-ly installation of methylpredisolon pulse therapyand plasma exchange therapy is effective in amilio-rating kidney and lung damages , while withdrawalof immunosuppresive drugs can cause the disease torelapse. Follow- up observations showed that anti-GBM disease is prognosticaly severe.