Background:Similar in histological characteristics and clinical manifestations, bronchiolitis obliterans syndrome (BOS) can develop following lung transplantation (LTx) or hematopoietic cell transplantation (HCT). In contrast to lung transplantation, where BOS is restricted to the lung allograft, HCT-related systemic graft-versus-host disease (GVHD) is the root cause of BOS. Because lung function declines following HCT, diagnosis becomes more difficult. Given the lack of proven effective medicines, treatment is based on empirical evidence.Methods:Cross-disciplinary learning is crucial, and novel therapies are under investigation to improve survival and avoid LTx. Recent advances have focused on updating the understanding of the etiology, clinical features, and pathobiology of BOS. It emphasizes the significance of learning from experts in other transplant modalities, promoting cross-disciplinary knowledge.Results:Our treatment algorithms are derived from extensive research and expert clinical input. It is important to ensure that immunosuppression is optimized and that any other conditions or contributing factors are addressed, if possible. Clear treatment algorithms are provided for each condition, drawing from the published literature and consensus clinical opinion. There are several novel therapies currently being investigated, such as aerosolized liposomal cyclosporine, Janus kinase inhibitors, antifibrotic therapies, and B-cell-directed therapies.Conclusions:We urgently need innovative treatments that can greatly increase survival rates and eliminate the need for LTx or re-transplantation.
背景：闭塞性细支气管炎综合征（BOS）在组织学特征和临床表现上相似，可发生于肺移植（LTx）或造血细胞移植（HCT）后。与肺移植中BOS仅限于移植肺不同，HCT相关的系统性移植物抗宿主病（GVHD）是BOS的根本原因。由于HCT后肺功能下降，诊断变得更加困难。鉴于缺乏经证实有效的药物，治疗主要基于经验性证据。 方法：跨学科学习至关重要，目前正在研究新的治疗方法以提高生存率并避免肺移植。近期的进展主要集中在更新对BOS病因、临床特征和病理生物学的理解。它强调了向其他移植领域的专家学习、促进跨学科知识交流的重要性。 结果：我们的治疗方案基于广泛的研究和专家临床意见。重要的是要确保免疫抑制得到优化，并在可能的情况下处理任何其他疾病或影响因素。根据已发表的文献和临床共识意见，为每种情况提供了明确的治疗方案。目前正在研究几种新型疗法，如雾化脂质体环孢素、Janus激酶抑制剂、抗纤维化疗法和B细胞导向疗法。 结论：我们迫切需要能够显著提高生存率并消除对肺移植或再次移植需求的创新治疗方法。
肿瘤(癌症)患者之家