Die pulmonale Manifestation der Sklerodermie

Abstract

The prognostically most decisive involvement of an organ in systemic sclerodermia is that of the lungs. Fibrotic alveolitis is seen on cellular examination. This results in a restrictive ventilatory disorder with the clinical signs of cough, exercise-induced dyspnoea, and even respiratory distress at rest. Roentgenologicaly the interstitial changes are mainly seen above the base of the lungs. Usually there is a finely reticulated, partly also nodular structure which may result in a final-stage lung that has undergone complete fibrotic metaplasia. Thin-layer computed tomography yields significantly more relevant information than the conventional x-ray. Besides fibrotic alveolitis, pulmonary complications arise also from aspirations. Pulmonary manifestations are mainly seen in male sclerodermia patients with an inflammatory course of the disease who are classified as suffering from type II or III of progressive systemic sclerodermia. Pulmonary manifestations can occur at all stages of the disease. However, diseases of the interstitial pulmonary tissue with early onset and rapid progression are particularly unfavourable. Patients in whom scl-70 antibodies are seen are more seriously involved than those without antibodies. If ACA antibodies are identified, a lower incidence of pulmonary complications may be expected. Treatment approaches with immunosuppressive therapy with cyclophosphamide and prednisone have been successful. Bronchoalveolar lavage helps to discover inflammatory processes in the periphery of the lung. This new knowledge results in recommending immunosuppressive treatment of patients in whom alveolitis has been diagnosed, especially if pulmonary function is seen to deteriorate.