Immunosuppression for the treatment of pulmonary hypertension in patients with systemic lupus erythematosus: A systematic review

PURPOSE: To conduct a systematic review with meta-analysis to determine the effects of immunosuppression on Group 1 Pulmonary Arterial Hypertension in patients with systemic lupus erythematosus (SLE). METHODS: We searched Medline, Embase, Web of Science, Clinicaltrials.gov, and Cochrane Central Register of Controlled Trials (CENTRAL) with a search strategy developed by a medical librarian. We included retrospective, cross-sectional, case-control, prospective studies, and randomized controlled trials (RCTs) in our analysis and only included studies that contained data for patients with SLE. We included any immunosuppressive agents (including but not limited to cyclophosphamide, glucocorticoids, mycophenolate mofetil, azathioprine, and rituximab) We assessed for risk of bias and certainty of evidence. Outcomes included hemodynamics (as measured by pulmonary arterial hypertension), functional status, 6 minute walk test (6MWT), quality of life, mortality, and serious adverse events. RESULTS: We included three studies. One RCT and two single-arm interventional observational studies. The RCT had a high risk of bias whereas the two single-arm interventional studies were graded as fair quality. Meta-analysis could not be conducted because of insufficient data. The RCT showed significant improvements in hemodynamics (as measured by pulmonary arterial pressures) and functional status. One observational study showed improvements in hemodynamics, functional status, and 6MWT. There were insufficient data for serious adverse events, mortality, and quality of life. CONCLUSIONS: Despite a high prevalence and with a poor prognosis, there is a paucity of data for the role of immunosuppression in the treatment of Group 1 Pulmonary Arterial Hypertension in SLE. More high-quality studies are needed, especially to investigate serious adverse events and quality of life.