| アブストラクト | 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. |
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| ジャーナル名 | Modern rheumatology |
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| Pubmed追加日 | 2023/9/25 |
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| 投稿者 | Atsumi, Tatsuya; Tan, Jin Yu; Chiang, Ashley Pei-Chun; Yu, Dae Young; Makanji, Yogeshwar; Wu, David Bin-Chia; Casorso, Jeremy; Kouhkamari, Mahsa; Lim, Sooyeol |
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| 組織名 | Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and;Graduate School of Medicine, Hokkaido University, Hokkaido, Japan.;Janssen Pharmaceutical Asia Pacific, Singapore.;Saw Swee Hock School of Public Health, National University of Singapore,;Singapore, Singapore.;Prospection Pty Ltd, Sydney, NSW, Australia.;Prospection KK, Tokyo, Japan. |
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| Pubmed リンク | https://www.ncbi.nlm.nih.gov/pubmed/37747781/ |
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