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Connective tissue diseases are a multisystem disorder that can cause impairments in quality of life, shorten life expectancy, and increase the risk of mortality at a younger age. These patients have an increased risk for the development of pulmonary hypertension through several mechanisms including pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease. This review aims to discuss the various presentations of connective tissue disease associated with pulmonary hypertension associated with interstitial lung disease, demographics, and survival. It gives an overview of accepted mechanisms of disease pathogenesis, discusses advances in diagnostics, and treatment options. Despite a deeper understanding of disease pathogenesis, treatment for this remains limited to prevention of disease progression. The identification of the primary disease driver requires careful evaluation of the disease phenotype and is a potential target for treatment and prevention of death.

Connective Tissue Disease, Interstitial Lung Disease, and Pulmonary Hypertension (CTD PH-ILD): A Distinct Entity and Potential Opportunity