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Introduction <p>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> Methods <p>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&#xa0;months of PAH diagnosis.</p> Results <p>The study included 141 patients (mean age 51 ± 16.7&#xa0;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&#xa0;months (<i>p</i> = 0.032 and <i>p</i> = 0.028), along with significant reductions in all-cause (<i>p</i> = 0.039) and PAH-related (<i>p</i> = 0.020) mortalities. No significant differences in deaths due to infections or malignancies were observed between groups.</p> Conclusion <p>Our data suggest that early initiation of IST may be associated with hemodynamic improvement; prospective confirmation in international cohorts is warranted.</p>

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Immunosuppressive Therapy for Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases: Insights from Japanese Registry

  • 期刊论文

  • 发表日期：

    2025年10月9日

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  • 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

摘要

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.