Pulmonary hypertension (PH) is associated with all of the connective tissue diseases (CTDs), but it is most commonly seen in systemic sclerosis (scleroderma), followed by systemic lupus erythematosus, and the antiphospholipid antibody syndrome. Although the prognosis for CTD-associated PH has improved with the availability of numerous pulmonary arterial hypertension therapies, it still carries a substantially poorer prognosis than idiopathic pulmonary arterial hypertension, and necessitates additional research into its treatment and prevention.
Further complicating treatment, CTD-associated PH is often multifactorial and can be caused by any of the World Health Organization categories of PH. In this issue of Advances in