Overview: Pulmonary Manifestations and Management

Abstract

Pulmonary complications of scleroderma (systemic sclerosis; SSc) are the single largest cause of disease-related mortality in a condition that has the highest death rate of any of the autoimmune rheumatic diseases. Fortunately, there has been recent progress in the area of assessment and treatment of both interstitial lung fibrosis and also pulmonary arterial hypertension, the two most important pulmonary manifestations of systemic sclerosis. There are many ways in which systemic sclerosis can affect the lungs, although the two most important complications are pulmonary arterial hypertension and interstitial fibrosis. Other complications include involvement of the chest wall skin and underlying connective tissue that may directly restrict respiratory excursion, muscle involvement with inflammation or fibrosis that impairs ventilation of the lungs.