Kumazaki S, Umeda Y, Sato K, Mishima H, Ishihara T, Uzawa T
Department of Respiratory Disease, Kanto Teishin Hospital, Tokyo, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1990 Apr;28(4):628-33.
This article describes a case of Goodpasture's syndrome controlled by double filtration plasmapheresis (DFPP) combined with steroid and immunosuppressant therapy. A 48-year-old male, clerk, complaining of fever, dry cough and macroscopic hematuria, was admitted to our hospital. Microscopic hematuria was first pointed out at age 40 on an annual check up. His laboratory data on admission revealed severe anemia, azothemia, macroscopic hematuria and proteinuria. His chest radiograph and CT revealed diffuse nodular densities in bilateral lung fields. Specimens obtained by transbronchial lung biopsy and open renal biopsy revealed linear deposition of IgG by direct immunofluorescent antibody methods. Circulating antiglomerular basement membrane antibody level determined with radioimmunoassay was 1.8% on admission, but one week later it elevated to 5.6% with progression of dyspnea, hypoxemia, and renal failure. Steroid pulse therapy and a total of 6 double filtration plasmaphereses were performed in the first month. Subsequently hypoxemia and dyspnea disappeared, and the chest radiograph of the 40th hospital day showed no abnormal shadows. Two months later recurrence of pulmonary hemorrhage was noticed. Immunosuppressant administration (Cyclophosphamide 100 mg/day) and a total of 10 DFPP procedures were performed with success. By DFPP, circulating anti-GBM antibody fell rapidly to within normal ranges, and anti-GBM antibody level elevated in removed plasma. We think DFPP is effective to remove circulating anti-GBM antibody in Goodpasture's syndrome.
本文描述了一例通过双重滤过血浆置换（DFPP）联合类固醇及免疫抑制剂治疗得以控制的肺出血肾炎综合征病例。一名48岁男性职员，因发热、干咳及肉眼血尿入院。其在40岁年度体检时首次被指出存在镜下血尿。入院时实验室检查数据显示有严重贫血、氮质血症、肉眼血尿及蛋白尿。胸部X线片及CT显示双侧肺野弥漫性结节状密度影。经支气管肺活检及开放性肾活检获取的标本通过直接免疫荧光抗体法显示IgG呈线性沉积。入院时采用放射免疫测定法测定的循环抗肾小球基底膜抗体水平为1.8%，但一周后随着呼吸困难、低氧血症及肾衰竭的进展，该水平升至5.6%。第一个月进行了类固醇冲击治疗及总共6次双重滤过血浆置换。随后低氧血症及呼吸困难消失，住院第40天的胸部X线片显示无异常阴影。两个月后发现肺出血复发。给予免疫抑制剂（环磷酰胺100mg/天）并总共进行了10次DFPP治疗，获得成功。通过DFPP，循环抗GBM抗体迅速降至正常范围内，且去除的血浆中抗GBM抗体水平升高。我们认为DFPP对于清除肺出血肾炎综合征中的循环抗GBM抗体有效。
