Pulmonary Hypertension Associated with Connective Tissue Disease

Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD); the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension (PAH) is dominantly associated with scleroderma, while postcapillary PH is most common in rheumatoid arthritis and lung disease-associated PH is typically found in myositis and sarcoidosis. Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the primary physicians providing the majority of care for this population, rheumatologists, need a good working knowledge of CTD-PH, its rather subtle presentation, and how to access the necessary investigations to screen for and identify patients with PH. The role of the rheumatologist does not stop at diagnosis; in some conditions such as lupus, optimizing immunosuppression is key to the management of PH, and unlike simple idiopathic PAH, the natural history of CTD-PH is often punctuated by complications of the CTD rather than just events due to progression of PH or therapy-related adverse events. The aim of this article is to provide an overview of all forms of CTD-PH, and to provide an easy reference source on current best practice.