Risk Factors for Adverse Outcomes in Connective Tissue Disease-Associated Pulmonary Hypertension

Pulmonary hypertension (PH) is a rare, life-threatening condition that can be associated with connective tissue disease (CTD). The incidence and prevalence of PH in CTD varies by disease, whereby certain disease manifestations are particularly associated with PH; nonetheless, once present, PH is almost uniformly a major driver of adverse outcomes. In this paper, the authors review the published literature on major CTDs, including systemic sclerosis and systemic lupus erythematosus, and summarize the risk factors for developing PH in each disease and risk factors for adverse outcomes and mortality among patients with CTD-PH. This review highlights the need for early diagnosis of PH in CTD and the impact of PH overlap syndromes on patient outcomes, providing the practicing clinician with a practical summary of CTD-PH.