Researchers reported that existing evidence supports the use of immunosuppressive therapeutics in connective tissue disease (CTD)-associated pulmonary arterial hypertension (PAH), especially in systemic lupus erythematosus-associated PAH (SLE-PAH), as published in the International Journal of Rheumatic Disease.

PAH is primarily characterized by pulmonary vascular remodeling which increases pulmonary vascular resistance. The current thinking when it comes to PAH therapeutics is that supportive therapy and vasodilators could help improve clinical outcomes. 

In the specific example of CTD-PAH, immunosuppressants have demonstrated efficacy in reversing pathology. However, because CTD-PAH is a complex disease, the therapeutic effects of immunosuppressive therapy have not been studied in full. Some patients also respond less well to immunosuppressants compared to others.

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The authors of this study conducted a systematic review to review studies that have assessed the use of immunosuppressive therapy to treat patients with CTD-PAH, as well as to investigate the factors that can influence the therapeutic potential of immunosuppressants.

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They conducted a literature search on academic search engines using keywords related to the topic investigated. The types of CTD included in this review included SLE, systemic scleroderma, and Sjögren’s syndrome. Immunosuppressants included cyclophosphamide, azathioprine, and mycophenolate mofetil. 

The research team included studies that were either case-control, case-series, cohort, cross-sectional design, or clinical trials. Patients must have had a diagnosis of CTD-PAH through validated standard diagnostic protocols. PAH was defined as mean pulmonary arterial pressure of more than 25 mm Hg and pulmonary capillary wedge pressure of less than 15 mm Hg. The researchers reviewed 9 studies that fit their inclusion criteria. 

“Overall, the literature supports the treatment effect of immunosuppressive therapy in CTD-PAH,” the authors wrote. “[Systemic scleroderma]-PAH patients responded poorly to immunosuppressive therapy, while SLE-PAH patients with better heart function or serositis at baseline showed better response.” 

The research team concluded that immunosuppressants have a significant therapeutic role in CTD-PAH, especially in SLE-PAH. 


Ding Y, Qian J, Zhang S, et al. Immunosuppressive therapy in patients with connective tissue disease-associated pulmonary arterial hypertension: a systematic reviewInt J Rheum Dis. Published online June 14, 2022. doi:10.1111/1756-185X.14368