[Idiopathic fibrosing alveolitis]

Abstract

The prognostic features of the disease are summed up. The leading symptom of the disease is the dyspnoe followed by a dry hacking cough, loss of weight and fever. Clinically we find rales, cyanosis clubbing and signs of right heart insufficiency. Lung function data point to a decreased vital capacity and diffusing capacity; residual volume and functional residual capacity are about normal. Corticosteroid monotherapy is not an efficient treatment. The combination of prednisolon + azathioprine improves vital capacity but not working capacity. Prednisolone and d-penicillamine improve all parameters including working capacity. This can be enhanced by still adding azathioprine or cyclophosphamide to prednisolone and d-penicillamine.