Jounieaux V, Néjjar O, Morinière P, Lévi-Valensi P, Fournier A
Service de Pneumologie, CHU, Hôpital Sud, Amiens.
Rev Mal Respir. 1993;10(1):49-52.
The authors report a case of a seventeen year old woman who was admitted to hospital for an extremely acute respiratory distress syndrome, which was revealed to be an exclusively pulmonary form of Goodpasture's syndrome. The positivity of the IgG immunofluorescence on open lung biopsy contrasted with the absence of circulating antibasement membrane antibody (ELISA). The combination of plasmapheresis, steroid therapy and cyclophosphamide enabled a rapidly favourable outcome and the patient could be weaned of the support therapy.
作者报告了一例17岁女性患者，该患者因极重度急性呼吸窘迫综合征入院，结果显示为古德帕斯彻综合征的单纯肺部形式。开胸肺活检时IgG免疫荧光呈阳性，而循环抗基底膜抗体（ELISA法）检测为阴性。血浆置换、类固醇疗法和环磷酰胺联合使用带来了迅速良好的疗效，患者得以停用支持治疗。
