Hamm H, Niedermeyer J, Krause J, Fabel H
Abteilung Pneumologie, Medizinische Hochschule Hannover.
Dtsch Med Wochenschr. 1992 May 29;117(22):858-62. doi: 10.1055/s-2008-1062386.
Anaemia (haemoglobin 10.7 g/dl) and small spotty infiltrates in both lungs were found in an 18-year-old man who had increasing haemoptysis over the preceding 3 weeks. Bronchoscopy revealed diffuse bilateral pulmonary haemorrhage. Further diagnostic measures provided no evidence of involvement of other organs, in particular the kidneys. The demonstration of anti-basement membrane antibodies confirmed the diagnosis of a disease within the group of immune-induced alveolar haemorrhage. The radiological signs in the lungs regressed over 3 weeks of administering prednisone, initially 100 mg daily, then 60 mg daily, and the patient was discharged. While being treated as an out-patient, with reduction of prednisone to 10 mg daily, the haemoptysis recurred so that a single dose of cyclophosphamide, 1.5 g, was added to the immunosuppressive treatment. There was no further haemoptysis and the prednisone was discontinued after having been given for 15 months. The patient has now been in complete clinical remission for 3 years and anti-basement membrane antibodies are no longer demonstrable. At no time was there any evidence of renal involvement in the sense of the classical Goodpasture's syndrome.
一名18岁男性，在过去3周内咯血症状加重，检查发现贫血（血红蛋白10.7g/dl），双肺有小片状浸润影。支气管镜检查显示双侧弥漫性肺出血。进一步的诊断措施未发现其他器官受累的证据，尤其是肾脏。抗基底膜抗体的检测证实了该疾病属于免疫性肺泡出血组。给予泼尼松治疗3周后，肺部的放射学征象消退，最初每日100mg，然后每日60mg，之后患者出院。在门诊治疗期间，泼尼松减至每日10mg时，咯血复发，因此在免疫抑制治疗中加用了1.5g环磷酰胺单次剂量。此后未再出现咯血，泼尼松在使用15个月后停药。患者目前已完全临床缓解3年，抗基底膜抗体也不再能检测到。在整个病程中，均未出现经典Goodpasture综合征意义上的肾脏受累证据。
