Gawryluk Dariusz, Baczkowska Teresa, Pazik Joanna, Wiatr Elzbieta, Pawłowski Jacek
III Kliniki Chorób Płuc.
Pneumonol Alergol Pol. 2005;73(1):79-84.
We describe the case of 24-years old man, smoking up to 60 cigarettes daily, with rapidly progressive crescentic glomerulonephritis in the course of Goodpasture's syndrome. The disease was initially presented with recurrent diffuse pulmonary hemorrhage with normal renal function and moderate proteinuria and haematuria on urinalysis lasting 2 months. Immunologic tests for ANCA and anti-GBM Ab were negative until the patient's renal function rapidly deteriorated during next 3 weeks. At the time of the diagnosis patient presented with renal insufficiency with oliguria requiring hemodialysis but without pulmonary hemorrhage. Renal biopsy showed cellular crescents in all glomeruli with linear deposition of IgG along the GBM. Repeated testing showed anti-GBM Ab. The patient received pulse cyclophosphamide, and pulse methylprednisolone continued by oral prednisone, and consecutive plasma exchange treatment but remained oliguric after 3 weeks of the treatment. The case confirm that in Goodpasture's syndrome even several days' delay in diagnosis and treatment has a strongly negative impact on outcome.
我们描述了一名24岁男性的病例，他每天吸烟多达60支，患有在Goodpasture综合征病程中快速进展的新月体性肾小球肾炎。该疾病最初表现为反复弥漫性肺出血，肾功能正常，尿液分析显示中度蛋白尿和血尿，持续2个月。抗中性粒细胞胞浆抗体（ANCA）和抗肾小球基底膜抗体（anti-GBM Ab）的免疫学检测均为阴性，直到患者的肾功能在接下来的3周内迅速恶化。在诊断时，患者出现肾功能不全伴少尿，需要进行血液透析，但无肺出血。肾活检显示所有肾小球均有细胞性新月体形成，IgG沿肾小球基底膜呈线性沉积。重复检测显示抗GBM抗体阳性。患者接受了环磷酰胺冲击治疗，继之以口服泼尼松维持的甲泼尼龙冲击治疗，以及连续的血浆置换治疗，但治疗3周后仍为少尿。该病例证实，在Goodpasture综合征中，即使诊断和治疗延迟数天也会对预后产生严重的负面影响。
