Pulmonary Hypertension Associated with Connective Tissue Disease

Pulmonary hypertension (PH) commonly complicates connective tissue disease and is associated with increased morbidity and mortality in this population. The incidence of PH varies widely between connective tissue diseases; patients with systemic sclerosis are most likely to develop PH. Several different types of PH can present in CTD, including PH related to left heart disease, respiratory disease such as interstitial lung disease or chronic obstructive lung disease, chronic thromboembolic disease, and renal disease. Importantly, CTD patients are at risk for developing pulmonary arterial hypertension (PAH), in which PH is present without any of the aforementioned co-morbidities. However, despite this increased risk of development of PH, patients with PAH may benefit from specific pulmonary vasodilator therapy and thus, are an important group to identify. Unfortunately, response to pulmonary vasodilator therapy in general is less robust and survival is worse in CTD-associated PAH than in other patient populations with PAH. Still, early diagnosis and treatment of PH, if indicated, may allow for institution of pulmonary vasodilator therapy and for appropriate referral for lung transplantation.