Takeuchi K, Takeda T, Sakai I, Taneichi K, Shibaki H
Department of Internal Medicine, Kitami Red Cross Hospital.
Ryumachi. 1997 Dec;37(6):781-7.
Goodpasture syndrome (GS) is an autoimmune disorder characterized by the association of pulmonary hemorrhage and rapidly progressive glomerulonephritis. The pathogenesis of GS is still unknown, but was shown to be the result that antibodies directed against glomerular basement membrane (GBM) antigens could injure both glomerular and pulmonary alveolar basement membrane. And membranous glomerulonephritis (MGN) is a glomerular disease characterized by epimembranous immune deposits and basement membrane thickening. MGN typically presents with the onset of nephrotic syndrome, but it often presents with only asymptomatic proteinuria. We reported an autopsy case of GS preceded with MGN. A 70-year-old man was admitted to our hospital with acute renal failure in May 2, 1996. Percutaneous renal biopsy demonstrated a crescentic glomerulonephritis associated with MGN and linear immunofluorescent staining of the basement membrane with antibodies to IgG. Two weeks later on admission he began to develop slight hemoptysis and chest X-ray showed pulmonary hemorrhage, Furthermore, his serum anti-GBM antibodies titer was very high. He was diagnosed as GS associated with MGN and treated with plasma exchange, glucocorticoid, and cyclophosphamide. Though his symptom was improved for intensive support, he suddenly died on June 22. Autopsied lungs showed focal pulmonary hemorrhage, but were not considered to be life-threatening. The cause of the death remained unclear.
肺出血肾炎综合征(GS)是一种自身免疫性疾病,其特征为肺出血和快速进展性肾小球肾炎。GS的发病机制尚不清楚,但已表明针对肾小球基底膜(GBM)抗原的抗体可损伤肾小球和肺泡基底膜。膜性肾小球肾炎(MGN)是一种以膜性免疫沉积物和基底膜增厚为特征的肾小球疾病。MGN通常以肾病综合征起病,但常仅表现为无症状蛋白尿。我们报告了一例先有MGN的GS尸检病例。一名70岁男性于1996年5月2日因急性肾衰竭入住我院。经皮肾活检显示为新月体性肾小球肾炎合并MGN,基底膜IgG抗体线性免疫荧光染色阳性。入院两周后,他开始出现轻微咯血,胸部X线显示肺出血,此外,他的血清抗GBM抗体滴度非常高。他被诊断为合并MGN的GS,并接受了血浆置换、糖皮质激素和环磷酰胺治疗。尽管经过强化支持治疗他的症状有所改善,但他于6月22日突然死亡。尸检发现肺部有局灶性肺出血,但不认为会危及生命。死亡原因仍不清楚。