Internal Medicine, Greenwich Hospital/Yale-New Haven Health, Greenwich, Connecticut, USA.
Medical Education, Greenwich Hospital/Yale-New Haven Health, Greenwich, Connecticut, USA
BMJ Case Rep. 2021 Feb 9;14(2):e239390. doi: 10.1136/bcr-2020-239390.
Goodpasture's syndrome is a rare vasculitis associated with anti-glomerular basement membrane (anti-GBM) autoantibodies that target type IV collagen found in the basement membranes of glomeruli and alveoli. We present a case of a 79-year-old man with seronegative Goodpasture's syndrome with predominant respiratory symptoms and mild acute kidney injury that initially improved. Final diagnosis was made by immunofluorescent staining on open lung biopsy which also revealed concomitant organising pneumonia. The patient underwent treatment with corticosteroids, cyclophosphamide, haemodialysis and plasmapheresis. This was an atypical presentation wherein the patient only exhibited pulmonary symptoms early in the course of illness in the setting of negative anti-GBM antibody serum testing, which made diagnosis challenging. With this case, we emphasise that clinicians should have a high suspicion for Goodpasture's syndrome in the setting of unexplained severe pulmonary or renal disease despite negative anti-GBM antibody testing.
Goodpasture 综合征是一种罕见的血管炎，与抗肾小球基底膜（anti-GBM）自身抗体有关，该自身抗体针对的是肾小球和肺泡基底膜中 IV 型胶原。我们报告了一例 79 岁男性，血清阴性 Goodpasture 综合征，主要表现为呼吸系统症状和轻度急性肾损伤，最初有所改善。最终诊断通过开胸肺活检的免疫荧光染色确定，该检查还显示同时存在机化性肺炎。患者接受了皮质类固醇、环磷酰胺、血液透析和血浆置换治疗。这是一种非典型表现，患者在疾病早期仅表现出肺部症状，抗 GBM 抗体血清检测阴性，这使得诊断具有挑战性。通过这个病例，我们强调，尽管抗 GBM 抗体检测阴性，临床医生在不明原因的严重肺部或肾脏疾病的情况下，应高度怀疑 Goodpasture 综合征。
