[Long-term follow-up of lung function parameters in patients with idiopathic pulmonary fibrosis treated with prednisone and azathioprin or d-penicillamine (author's transl)]

Abstract

The prognosis of idiopathic pulmonary fibrosis is poor, it amounts to 4-6 years (after beginning of the symptoms). Spontaneous remissions are very rare, the corticosteroid monotherapy shows a certain success in only 11-16-20% of the cases. Therefore, since 1970 we carry out a combined therapy (see above); the results, confirmed by pulmonary function tets (vital capacity, static compliance, pO2 at rest and after exercise) will be presented. In summary: (1) Azathioprin and Prednisone: After a treatment of 2 years: 6 patients improved, 2 deteriorated, 3 died; the corresponding figures after 4 years were 6/1/4, after 6 years 3/-/5, after 8 years 2/-/3; (2) D-Penicillamine and Prednisone: After 2 years 3/2/4, 1 unchanged; after 4 years 1/-/4, 1 unchanged. The results of the therapy are discussed especially regarding prognosis of the disease and used drugs.