Overview of lung involvement: Diagnosis, differential diagnosis and monitoring

Abstract

Lung complications have the highest mortality of any of the disease-related complications of systemic sclerosis following improvements in outcome of scleroderma renal crisis. Currently these complications are spread between lung fibrosis and pulmonary arterial hypertension, with a particularly poor survival when both of these lung processes occur concurrently. Since there is symptomatic overlap between the lung complications and other manifestations of SSc that lead to breathlessness and limited exercise capacity, the differential diagnosis of pulmonary complications is important. In addition, therapeutic options for lung complications have increased as evidence supporting current treatment strategies grows; new options for the treatment of pulmonary arterial hypertension emerge and potential use of antifibrotic agents that are now licensed for the treatment of idiopathic pulmonary fibrosis for other forms of lung fibrosis such as systemic sclerosis.