Maes B, Vanwalleghem J, Kuypers D, Van Damme B, Waer M, Vanrenterghem Y
Departments of Nephrology and Pathology, University Hospital, Leuven B-3000, Gasthuisberg, Belgium.
Am J Kidney Dis. 1999 Feb;33(2):E3. doi: 10.1016/s0272-6386(99)70324-0.
Antiglomerular basement membrane (anti-GBM) disease is characterized by a linear deposition of immunoglobulins along the glomerular basement membrane. A 67-year-old man with a recently discovered monoclonal gammopathy of unknown significance (MGUS) presented with microscopic hematuria, nephrotic-range proteinuria, and rapidly deteriorating renal function after a pneumonia. Renal histology showed a crescentic glomerulonephritis; immunohistology showed intense linear staining of the GBM with immunoglobulin A (IgA) and moderate linear staining with kappa and lambda light chains. Screening for systemic disease, including diabetes mellitus, lupus erythematodes disseminatus, cryoglobulinemia, was negative. Serological tests for detection of anti-GBM antibodies were positive for IgA class and negative for IgG. Further examination indicated a bronchial carcinoma T2N2M0. This clinical report adds new information to the spectrum of anti-GBM disease and suggests that neoplasia may be associated with unusual exposure of and/or immune response to epitopes in the GBM.
抗肾小球基底膜（anti-GBM）病的特征是免疫球蛋白沿肾小球基底膜呈线性沉积。一名67岁男性，近期发现意义未明的单克隆丙种球蛋白病（MGUS），在患肺炎后出现镜下血尿、肾病范围蛋白尿及肾功能迅速恶化。肾脏组织学检查显示为新月体性肾小球肾炎；免疫组织学检查显示肾小球基底膜（GBM）有强烈的免疫球蛋白A（IgA）线性染色以及κ和λ轻链的中度线性染色。对包括糖尿病、系统性红斑狼疮、冷球蛋白血症在内的全身性疾病筛查均为阴性。检测抗GBM抗体的血清学试验显示IgA类阳性，IgG类阴性。进一步检查提示为支气管癌T2N2M0。本临床报告为抗GBM病谱增添了新信息，并提示肿瘤可能与GBM中表位的异常暴露和/或免疫反应有关。
