Recognizing pulmonary complications in patients with systemic sclerosis

Interstitial lung disease is the most common pulmonary complication in patients with systemic sclerosis (SSc). Typical symptoms are dry cough, fatigue, and progressive dyspnea on exertion. Chest radiographic and high-resolution CT findings include peripheral, basilar reticulonodular changes and honeycombing. Treatment is usually with cyclophosphamide; alternatives include azathioprine and corticosteroids. Pulmonary arterial hypertension (PAH) also is common in SSc. Patients with mild PAH who demonstrate considerable vasoreactivity are potential candidates for treatment with oral calcium channel blockers. Select patients may be given slow-release oral nifedipine, 30 mg/d, with the dosage slowly increased as tolerated. Other therapies that can reduce symptoms and improve exercise tolerance and hemodynamics include bosentan and epoprostenol.