Serisier D J, Wong R C W, Armstrong J G
Department of Respiratory Medicine, Mater Adult Hospital, Raymond Tce, South Brisbane, Queensland 4101, Australia.
Thorax. 2006 Jul;61(7):636-9. doi: 10.1136/thx.2004.028985.
Anti-glomerular basement membrane (anti-GBM) disease represents the spectrum of disease attributable to circulating anti-GBM antibodies. While active anti-GBM disease in the absence of circulating anti-GBM antibodies has been described, it is considered rare with the use of current routinely available assays. We report four subjects with features consistent with active anti-GBM antibody disease without detectable antibodies by routinely available enzyme linked immunosorbent assay (ELISA) and immunoblot techniques. All were smokers who presented with diffuse alveolar haemorrhage, minimal renal involvement, and undetectable anti-GBM antibodies. Seronegative anti-GBM disease with predominant pulmonary involvement may be more common than previously appreciated and should be part of the differential diagnosis for otherwise unexplained diffuse alveolar haemorrhage. Renal biopsy with immunofluorescent studies should be considered in the diagnostic evaluation of such subjects, including those with idiopathic pulmonary haemosiderosis.
抗肾小球基底膜（anti-GBM）病代表了由循环抗GBM抗体引起的一系列疾病。虽然已经描述了在没有循环抗GBM抗体的情况下出现的活动性抗GBM病，但使用当前常规可用的检测方法时，这种情况被认为很罕见。我们报告了4例具有与活动性抗GBM抗体病一致特征的患者，通过常规可用的酶联免疫吸附测定（ELISA）和免疫印迹技术未检测到抗体。所有患者均为吸烟者，表现为弥漫性肺泡出血、轻微肾脏受累且未检测到抗GBM抗体。以肺部受累为主的血清阴性抗GBM病可能比以前认识到的更为常见，对于原因不明的弥漫性肺泡出血，应将其纳入鉴别诊断。对于此类患者，包括特发性肺含铁血黄素沉着症患者，在诊断评估中应考虑进行免疫荧光研究的肾活检。
