Different therapies and factors influencing response to therapy in idiopathic diffuse fibrosing alveolitis.

The success of 3 different treatment regimens was compared in 15 patients with diffuse fibrosing alveolitis. Improvement or deterioration of lung function (vital capacity (VC) and PaO2 changes during exercise) were used as a control of therapy. The combination of prednisolone and azathioprine proved to be most effective: in 4 of 6 patients both VC and PaO2 improved. Combinations with D-penicillamine resulted in a high number of unwanted side effects: in 5 of 13 treated patients it had to be withdrawn. 6 patients were treated with prednisolone alone; only 1 of them improved. Patients with a short duration of symptoms prior to therapy and initially mild impairment of lung function showed a higher degree of improvement during therapy. The consequence for the management of lung fibrosis is to establish diagnosis and treatment as early as possible.