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.
Keywords: Prevalence; pulmonary arterial hypertension; systemic lupus erythematosus; treatment patterns.
© Japan College of Rheumatology 2023. Published by Oxford University Press.