Pulmonary Hypertension Associated with Connective Tissue Disease

PH remains a common complication of CTD and can present in various forms, most commonly in the setting of left heart disease, lung disease, or PAH. The presence of PH in any form portends a poor outcome. Advances in screening for PAH in high-risk cohorts, such as SSc, along with increased awareness of treatment options for non-PAH forms of PH may help support efforts to identify pulmonary vascular disease earlier in this at-risk population and allow for intervention. Initial combination therapy is now recommended as standard of care for treatment-naive, intermediate-risk patients with PAH-CTD, and pulmonary vasodilator therapy is approved for both patients with CTEPH and patients with PH-ILD with CTD. Furthermore, lung transplant remains an option in the appropriate patient. Still, despite these advances, there remains an ongoing need to improve outcomes for patients with CTD with PH.