Immunosuppressive Therapy for Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases: Insights from Japanese Registry.
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Tamura Yudai, Tamura Yuichi, Takemura Ryo, Taniguchi Yu, Tsujino Ichizo, Inami Takumi, Matsubara Hiromi, Shigeta Ayako, Hatano Masaru, Adachi Shiro, Tahara Nobuhiro, Sakurai Keiichi, Horimoto Koshin, Yaoita Nobuhiro, Abe Kohtaro, Dohi Yoshihiro, Kimura Kazuhiro, Kubota Kayoko, Kikuchi Noriko, Yasuoka Hidekata, Baba Yuichi, Shinke Toshiro, Amino Mari, Yamaguchi Natsumi, Ikeda Satoshi, Sato Teruki, Ishida Masaru, Sera Fusako, Nakanishi Naohiko, Konishi Hakuoh, Kinugawa Koichiro, Kashimura Takeshi, Dohi Kaoru, Nakamura Kazufumi, Usui Soichiro, Tanaka Sumiaki, Kubota Shuji, Ikeda Nobutaka, Yoshikawa Masanori, Odagiri Keiichi, Tasaka Sadatomo, Takeishi Yasuchika, Sugano Teruyasu, Sugimura Koichiro, Tatsumi Koichiro, Kuwana Masataka, Japan Pulmonary Hypertension Registry Network
Аннотация
METHODS: Clinical data for patients with non-SSc CTD-PAH were collected from the Japan Pulmonary Hypertension Registry spanning 2008-2021. Early IST was defined as the initiation or intensification of therapy within 3 months of PAH diagnosis.
RESULTS: The study included 141 patients (mean age 51 ± 16.7 years; 95% female), with 57 receiving early IST, across 43 centers in Japan. The primary underlying diseases were systemic lupus erythematosus, mixed connective tissue disease, and Sjögren syndrome. At baseline, there were no significant differences in hemodynamics or PAH treatment regimens between the IST and non-IST groups. However, the IST group was notably younger, had higher plasma IgG levels, and maintained better renal function. The IST group showed significantly greater improvements in mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) over 12 months (p = 0.032 and p = 0.028), along with significant reductions in all-cause (p = 0.039) and PAH-related (p = 0.020) mortalities. No significant differences in deaths due to infections or malignancies were observed between groups.
CONCLUSION: Our data suggest that early initiation of IST may be associated with hemodynamic improvement; prospective confirmation in international cohorts is warranted.