{"title":"免疫抑制治疗与结缔组织疾病相关的肺动脉高压：来自日本注册的见解","authors":"Yudai Tamura, Yuichi Tamura, Ryo Takemura, Yu Taniguchi, Ichizo Tsujino, Takumi Inami, Hiromi Matsubara, Ayako Shigeta, Masaru Hatano, Shiro Adachi, Nobuhiro Tahara, Keiichi Sakurai, Koshin Horimoto, Nobuhiro Yaoita, Kohtaro Abe, Yoshihiro Dohi, Kazuhiro Kimura, Kayoko Kubota, Noriko Kikuchi, Hidekata Yasuoka, Yuichi Baba, Toshiro Shinke, Mari Amino, Natsumi Yamaguchi, Satoshi Ikeda, Teruki Sato, Masaru Ishida, Fusako Sera, Naohiko Nakanishi, Hakuoh Konishi, Koichiro Kinugawa, Takeshi Kashimura, Kaoru Dohi, Kazufumi Nakamura, Soichiro Usui, Sumiaki Tanaka, Shuji Kubota, Nobutaka Ikeda, Masanori Yoshikawa, Keiichi Odagiri, Sadatomo Tasaka, Yasuchika Takeishi, Teruyasu Sugano, Koichiro Sugimura, Koichiro Tatsumi, Masataka Kuwana","doi":"10.1007/s12325-025-03389-z","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>The combination of immunosuppressive therapy (IST) and pulmonary artery vasodilators has demonstrated potential effectiveness in treating pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD-PAH) other than systemic sclerosis (SSc). However, large-scale studies of this topic are limited. This study aimed to evaluate the effectiveness of early IST in treating PAH in patients diagnosed with non-SSc CTD-PAH.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>Our data suggest that early initiation of IST may be associated with hemodynamic improvement; prospective confirmation in international cohorts is warranted.</p>","PeriodicalId":7482,"journal":{"name":"Advances in Therapy","volume":" ","pages":""},"PeriodicalIF":4.0000,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Immunosuppressive Therapy for Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases: Insights from Japanese Registry.\",\"authors\":\"Yudai Tamura, Yuichi Tamura, Ryo Takemura, Yu Taniguchi, Ichizo Tsujino, Takumi Inami, Hiromi Matsubara, Ayako Shigeta, Masaru Hatano, Shiro Adachi, Nobuhiro Tahara, Keiichi Sakurai, Koshin Horimoto, Nobuhiro Yaoita, Kohtaro Abe, Yoshihiro Dohi, Kazuhiro Kimura, Kayoko Kubota, Noriko Kikuchi, Hidekata Yasuoka, Yuichi Baba, Toshiro Shinke, Mari Amino, Natsumi Yamaguchi, Satoshi Ikeda, Teruki Sato, Masaru Ishida, Fusako Sera, Naohiko Nakanishi, Hakuoh Konishi, Koichiro Kinugawa, Takeshi Kashimura, Kaoru Dohi, Kazufumi Nakamura, Soichiro Usui, Sumiaki Tanaka, Shuji Kubota, Nobutaka Ikeda, Masanori Yoshikawa, Keiichi Odagiri, Sadatomo Tasaka, Yasuchika Takeishi, Teruyasu Sugano, Koichiro Sugimura, Koichiro Tatsumi, Masataka Kuwana\",\"doi\":\"10.1007/s12325-025-03389-z\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>The combination of immunosuppressive therapy (IST) and pulmonary artery vasodilators has demonstrated potential effectiveness in treating pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD-PAH) other than systemic sclerosis (SSc). However, large-scale studies of this topic are limited. This study aimed to evaluate the effectiveness of early IST in treating PAH in patients diagnosed with non-SSc CTD-PAH.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>Our data suggest that early initiation of IST may be associated with hemodynamic improvement; prospective confirmation in international cohorts is warranted.</p>\",\"PeriodicalId\":7482,\"journal\":{\"name\":\"Advances in Therapy\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":4.0000,\"publicationDate\":\"2025-10-09\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Advances in Therapy\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s12325-025-03389-z\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"MEDICINE, RESEARCH & EXPERIMENTAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Advances in Therapy","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s12325-025-03389-z","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"MEDICINE, RESEARCH & EXPERIMENTAL","Score":null,"Total":0}
Immunosuppressive Therapy for Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases: Insights from Japanese Registry.
Introduction: The combination of immunosuppressive therapy (IST) and pulmonary artery vasodilators has demonstrated potential effectiveness in treating pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD-PAH) other than systemic sclerosis (SSc). However, large-scale studies of this topic are limited. This study aimed to evaluate the effectiveness of early IST in treating PAH in patients diagnosed with non-SSc CTD-PAH.
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.
期刊介绍：
Advances in Therapy is an international, peer reviewed, rapid-publication (peer review in 2 weeks, published 3–4 weeks from acceptance) journal dedicated to the publication of high-quality clinical (all phases), observational, real-world, and health outcomes research around the discovery, development, and use of therapeutics and interventions (including devices) across all therapeutic areas. Studies relating to diagnostics and diagnosis, pharmacoeconomics, public health, epidemiology, quality of life, and patient care, management, and education are also encouraged.
The journal is of interest to a broad audience of healthcare professionals and publishes original research, reviews, communications and letters. The journal is read by a global audience and receives submissions from all over the world. Advances in Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. Submissions are welcomed whether they relate to an international and/or a country-specific audience, something that is crucially important when researchers are trying to target more specific patient populations. This inclusive approach allows the journal to assist in the dissemination of all scientifically and ethically sound research.
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