Systemic sclerosis associated interstitial lung disease

The involvement of the lungs in systemic sclerosis (SSc) is one of the most frequent organ manifestations and is afrequent cause of death among these patients. The course of SSc can be either stable or show slow or rapid progressive deterioration. In particular, interstitial lung disease (ILD) is associated with a poor prognosis and a high mortality and disability. The SSc-ILD often develops during the early phases of the disease. The timely diagnosis of the disease is of vital importance for survival of these patients. The most important indications for the diagnosis of SSc-ILD are the clinical presentation of the patient usually with coughing and progressive dyspnea, deterioration of lung function, the autoantibody constellation in addition to radiological imaging with thorax computed tomography (CT). Despite advances in treatment of SSc in general, there are still only limited specific possibilities for treatment of SSc-ILD, mostly relying on immunosuppression and other pharmacological options as well as lung and stem cell transplantation in selected cases. In severe cases the treatment strategy should be individualized in centers of expertise based on the disease course and the individual needs of the patient