Uezu Y, Kiyatake I, Tokuyama K
Department of Internal Medicine, Urasoe General Hospital, Okinawa, Japan.
Nihon Jinzo Gakkai Shi. 1999 Aug;41(5):499-504.
A 22-year-old woman was admitted to our hospital for evaluation of fever, renal dysfunction, and a 3-month-history of macrohematuria. Laboratory evaluation revealed proteinuria (1.8 g/day), hypoproteinemia, microcytic microchromic anemia, renal failure (blood urea nitrogen 30.3 mg/dl, serum creatinine 4.0 mg/dl), and positive serum antiglomerular basement membrane (anti-GBM) antibody. Renal biopsy revealed cellular crescents in all 8 glomeruli and partial rupture of the GBM. The interstitium showed severe inflammatory cell infiltration. Immunofluorescent examination revealed linear deposits of IgG and C3 along the GBM. Pulmonary biopsy revealed linear deposits of IgG along the alveolar basement membrane in the immunofluorescent examination. A diagnosis of Goodpasture's syndrome was made because all of the diagnostic criteria were fulfilled. After admission, the patient's renal function deteriorated rapidly. Hemodialysis was started, and the patient was treated with methylprednisolone pulse therapy and oral prednisolone with double filtration plasma pheresis (DFPP). However, her renal function did not improve. On the 30th hospital day, she showed hemoptysis, and a chest X-ray and CT revealed massive bilateral pulmonary hemorrhage. Despite treatment with pulsed methylprednisolone, oral prednisolone (80 mg/day), and DFPP, the pulmonary hemorrhage improved only transiently, worsening again 5 days later. Cyclophosphamide pulse therapy was administered. After this treatment, the patient's pulmonary manifestations and pulmonary hemorrhage improved. At the present time she is on maintenance dialysis therapy without pulmonary manifestations. These findings suggest that cyclophosphamide pulse therapy is effective against Goodpasture's syndrome with massive pulmonary hemorrhage showing resistance to other conventional therapy.
一名22岁女性因发热、肾功能不全及3个月的肉眼血尿病史入院。实验室检查发现蛋白尿（1.8克/天）、低蛋白血症、小细胞低色素性贫血、肾衰竭（血尿素氮30.3毫克/分升，血清肌酐4.0毫克/分升），血清抗肾小球基底膜（抗GBM）抗体阳性。肾活检显示所有8个肾小球均有细胞性新月体形成，GBM部分断裂。间质有严重的炎性细胞浸润。免疫荧光检查显示IgG和C3沿GBM呈线性沉积。肺活检在免疫荧光检查中显示IgG沿肺泡基底膜呈线性沉积。由于满足所有诊断标准，故诊断为Goodpasture综合征。入院后，患者肾功能迅速恶化。开始进行血液透析，并给予患者甲泼尼龙冲击治疗、口服泼尼松龙及双重滤过血浆置换（DFPP）。然而，她的肾功能并未改善。住院第30天，她出现咯血，胸部X线和CT显示双侧大量肺出血。尽管给予甲泼尼龙冲击治疗、口服泼尼松龙（80毫克/天）及DFPP，肺出血仅短暂改善，5天后再次恶化。给予环磷酰胺冲击治疗。经过该治疗，患者的肺部表现及肺出血得到改善。目前她接受维持性透析治疗，无肺部表现。这些发现提示环磷酰胺冲击治疗对伴有大量肺出血且对其他传统治疗耐药的Goodpasture综合征有效。
