Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, R.O.C.
Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark.
In Vivo. 2024 Nov-Dec;38(6):3117-3124. doi: 10.21873/invivo.13797.
BACKGROUND/AIM: Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a severe complication characterized by elevated pulmonary artery pressure, which can lead to right heart failure and death, if untreated. Standard treatments often fail to adequately manage symptoms, highlighting the need for novel therapeutic approaches. This study investigated the efficacy of molecular hydrogen (H) therapy in a patient with CTD-PAH.
We present the case of a 56-year-old female with CTD-PAH, diagnosed in 2013 with Sjogren's syndrome complicated by interstitial lung disease (ILD) and PAH. Despite treatment with sildenafil, bosentan, macitentan, iloprost, and corticosteroids, her condition deteriorated, resulting in severe dyspnea and cardiogenic shock in 2020. In May 2023, molecular hydrogen therapy was initiated as an adjuvant treatment. The patient received daily hydrogen capsules, which led to increased CD127+ Treg cells, reduced anti-Ro antibodies, and decreased B cell subsets. Her clinical symptoms stabilized without adverse effects.
This case highlights the potential benefits of molecular hydrogen therapy in CTD-PAH. H therapy exhibiting anti-inflammatory and immunomodulatory effects, leading to improved immune cell profiles and stabilizing clinical symptoms in a patient unresponsive to conventional treatments. Further research is needed to elucidate the mechanisms of H therapy and validate its efficacy in larger cohorts. Molecular hydrogen therapy shows promise as a safe adjunctive treatment for CTD-PAH, offering a new approach for managing this challenging condition.
背景/目的：结缔组织病相关性肺动脉高压（CTD-PAH）是一种严重的并发症，其特征是肺动脉压升高，如果不治疗，可导致右心衰竭和死亡。标准治疗方法往往无法充分缓解症状，凸显出需要新的治疗方法。本研究调查了分子氢（H）治疗在 CTD-PAH 患者中的疗效。
我们报告了一例 56 岁女性 CTD-PAH 患者，2013 年诊断为干燥综合征并发间质性肺病（ILD）和 PAH。尽管接受了西地那非、波生坦、马昔腾坦、伊洛前列素和皮质类固醇治疗，但病情仍恶化，2020 年出现严重呼吸困难和心源性休克。2023 年 5 月，开始采用分子氢治疗作为辅助治疗。患者每日服用氢胶囊，导致 CD127+Treg 细胞增加，抗 Ro 抗体减少，B 细胞亚群减少。她的临床症状稳定，无不良反应。
本病例强调了分子氢治疗在 CTD-PAH 中的潜在益处。H 治疗具有抗炎和免疫调节作用，可改善免疫细胞谱，并稳定对常规治疗无反应的患者的临床症状。需要进一步研究阐明 H 治疗的机制，并在更大的队列中验证其疗效。分子氢治疗有望成为 CTD-PAH 的安全辅助治疗方法，为治疗这种具有挑战性的疾病提供了新方法。
