Lung disease is a frequent manifestation in patients with systemic sclerosis, and has replaced renal disease as the leading cause of mortality. The two major types of pulmonary diseases in systemic sclerosis are interstitial lung disease (ILD), occurring in approximately 80% of patients, and pulmonary hypertension (PH), occurring in up to 50% of patients. Prognostic factors for poor outcomes include male sex, the presence of lung involvement early in disease, low Dlco, severe Raynaud’s phenomenon, and cigarette smoking.
ILD may occur in either limited or diffuse cutaneous scleroderma. Retrospective autopsy studies show that clinical determinants of pulmonary disease may be relatively insensitive. Diagnosis of ILD is made using history, physical examonation, chest X ray, HRCT, BAL and pulmonary function test. DLco is the best index of the extent of the ILD when compared with HRCT as the “gold standard”. Based on the concept that persistent alveolitis is associated with a significant reduction of lung volumes and DLco, several anti-inflammatory drugs have been used in the treatment of established scleroderma lung fibrosis.
Although corticosteroids have no apparent effect in the treatment of pulmonary fibrosis, when used at an early stage of the disease, they improve pulmonary function, alveolitis and reduce mortality; however, high-dose glucocorticoids probably should be avoided in scleroderma due to the associated risk of scleroderma renal crisis. Colchicine, D-Penicillamine and chlorambucil are also used but the best results have been observed with cyclophosphamide.