Fukazawa M, Kawano M, Hisano S, Ueda K, Matsuba K
Department of Paediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Eur J Pediatr. 1990 Mar;149(6):441-2. doi: 10.1007/BF02009669.
In an 11-year-old girl with severe dyspnoea and cyanosis, idiopathic pulmonary fibrosis (IPF) was diagnosed. The PaO2 of blood could be maintained between 50 mm Hg and 70 mm Hg with a high dose pulse corticosteroid therapy, although this dose could not be reduced. Azathioprine in addition to prednisolone provoked deterioration. Twelve weeks after admission, cyclosporin A (Cs A), a known immunosuppressant, was started together with prednisolone. The PaO2 reverted to normal within 1 month and prednisolone could be tapered off within 6 months. Two years after admission, the patient is free of symptoms on continuous Cs A therapy. This case seems to be the first case successfully treated with Cs A.
在一名患有严重呼吸困难和发绀的11岁女孩中，诊断出特发性肺纤维化（IPF）。尽管高剂量脉冲皮质类固醇疗法的剂量无法降低，但血液中的PaO2可维持在50毫米汞柱至70毫米汞柱之间。除泼尼松龙外，硫唑嘌呤会引发病情恶化。入院12周后，开始使用已知的免疫抑制剂环孢素A（CsA）联合泼尼松龙治疗。PaO2在1个月内恢复正常，泼尼松龙可在6个月内逐渐减量。入院两年后，患者在持续使用CsA治疗的情况下无症状。该病例似乎是首例成功使用CsA治疗的病例。
