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

Abstract

ABSTRACT Objectives 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. Methods 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. Results 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 <1 year from SLE diagnosis. SLE–PAH patients were predominantly females (88% vs. 72%), had a lower mean age at SLE diagnosis (53 vs. 56 years), and had more severe SLE (61% vs. 25%) than non-PAH–SLE patients. Glucocorticoids (58%) and vasodilators (27%) were preferred first-line monotherapy for SLE–PAH. A combination of glucocorticoids and immunosuppressants (19%) was the predominant first-line combination therapy. Endothelin receptor antagonists (40% and 44%) and nitric oxide analogues (31% and 40%) were dominant first- and second-line vasodilators. Conclusions SLE–PAH patients were predominantly females, were younger at diagnosis, and had more severe SLE than non-PAH–SLE patients. Most were diagnosed <1 year of SLE diagnosis. In Japan’s real-world practice, the initial treatment goal is SLE management, while vasodilators are preferred in advanced diseases, as per the Medical Data Vision database.