[Interstitial lung disease (ILD) in systemic sclerosis (SSc)]

Abstract

Intersitial lung disease is a frequent complication of systemic sclerosis that often has a poor pognosis and together with pulmonary arterial hypertension are the most common cause of death in scleroderma patients. For detection and evaluation of interstitial lung disease, high-resolution CT and pulmorary functional tests are pivotal. The decision about whether to start treatment is often the most difficult challenge. Patients with short duration of systemic disease with recent deterioration in DCO are the candidates for immunosupressive therapy. Best current initial treatment is intravenous monthly cyclophosphamide together with low-dose oral glucocorticoids although azathioprine and mycophenolate mofetil are also widely used.