University of Toledo Medical Center, Toledo, OH, USA.
Nephrol Dial Transplant. 2010 Oct;25(10):3446-9. doi: 10.1093/ndt/gfq437. Epub 2010 Jul 21.
Goodpasture's disease is associated with circulating anti-glomerular basement membrane (anti-GBM) antibodies. Thrombotic thrombocytopenic purpura (TTP) is a disease related to platelet clumping and microthrombosis in the circulation. We report an unusual case where both entities coexist in the same patient. The patient was a 43-year-old Caucasian male, with a recent history of inhalational hydrocarbon exposure for ~10 weeks. He initially presented with confusion, persistent fever and acute oliguric renal failure. In addition, he was found to be thrombocytopenic and had concurrent microangiopathic haemolytic anaemia. All presenting signs, symptoms and laboratory findings had a temporal relationship within 3 weeks. In addition, he was also found to have active pulmonary hemorrhage and positive anti-GBM antibody. During his stay, the patient underwent treatment with plasmapheresis, and an open lung biopsy, which confirmed the diagnosis of anti-GBM disease. This case report confirms previously reported findings which were noted in a few sporadic case reports about the possible association between Goodpasture's disease and TTP. In addition, it adds to our current understanding of the pathophysiology of autoimmune diseases in general and supports the theory of an autoimmune mosaic, which has also been noted in various other autoimmune diseases.
Goodpasture 病与循环抗肾小球基底膜（anti-GBM）抗体有关。血栓性血小板减少性紫癜（TTP）是一种与血小板聚集和循环中的微血栓形成有关的疾病。我们报告了一个不常见的病例，其中两种疾病同时存在于同一患者中。患者为 43 岁白人男性，有最近吸入碳氢化合物暴露史约 10 周。他最初表现为意识障碍、持续发热和急性少尿性肾衰竭。此外，他还被发现血小板减少，同时伴有微血管性溶血性贫血。所有表现出的症状、体征和实验室发现都在 3 周内具有时间相关性。此外，他还被发现有活动性肺出血和抗 GBM 抗体阳性。在住院期间，患者接受了血浆置换和开胸肺活检，这证实了抗 GBM 疾病的诊断。本病例报告证实了以前在少数散发性病例报告中观察到的关于 Goodpasture 病和 TTP 之间可能存在关联的发现。此外，它增加了我们对自身免疫性疾病一般发病机制的理解，并支持自身免疫马赛克理论，该理论也在各种其他自身免疫性疾病中得到了证实。
