Prevalence, patient characteristics and treatment patterns among systemic lupus erythematosus-pulmonary arterial hypertension patients in real-world clinical practice: A retrospective analysis of Medical Data Vision Database in Japan

Real-world evidence regarding prevalence, patient characteristics, and treatment patterns for pulmonary arterial hypertension (PAH) related to systemic lupus erythematosus (SLE) in Japan is limited. We conducted a retrospective study analysing Japan's Medical Data Vision database from April 2008 to September 2020. Prevalence, incidence, patient characteristics, treatment patterns, and use of vasodilators by treatment line were evaluated. The prevalence of PAH was 0.392% in SLE patients (n = 114/29,077). Cumulative incidence was 0.53% (3 years) and 0.77% (5 years). Of 114 SLE-PAH patients, 49% developed PAH