Efficacy of cyclosporin A for idiopathic pulmonary fibrosis

Abstract

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.