Connective tissue disease-related interstitial lung disease and pulmonary hypertension.

Abstract

Pulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTDs) and may develop either in isolation or in combination with interstitial lung disease (ILD). Based on the World Health Organization PH classification into five groups, patients with CTDs typically belong either to group 1 pulmonary arterial hypertension or to group 3 PH due to chronic lung disease and hypoxaemia (PH-ILD). Recent epidemiological studies have delineated the phenotypical complexity of CTD patients who present with both PH and ILD. This review explores the prevalence, diagnosis, pathophysiology, survival and management strategies for CTD-ILD-PH.