Luo Caikang, Shi Jiang, Zhang Jiaqin, Lin Yanwei, Pan Yining, Zhang Jie, Yang Chao, Peng Guilin, He Jianxing, Xu Xin
Department of Organ Transplantation, and Department of Thoracic Surgery and Oncology, the First Affiliated Hospital of Guangzhou Medical University, State Key Laboratory of Respiratory Disease and National Clinical Research Center for Respiratory Disease, Guangzhou, China.
First Clinical College, Guangzhou Medical University, Guangzhou, China.
Clin Exp Rheumatol. 2025 Mar;43(3):477-485. doi: 10.55563/clinexprheumatol/tjnyz5. Epub 2025 Jan 14.
The progressive decline in interstitial lung disease associated with non-scleroderma connective tissue disease (ILD-NSCTD) is linked to poor prognosis and frequently results in respiratory failure. Lung transplantation (LTx) offers a viable treatment option, yet its outcomes in ILD-NSCTD remain contentious, particularly across different subtypes.
This retrospective cohort study included patients with idiopathic pulmonary fibrosis (IPF) (n=11,610) and ILD-NSCTD (n=610) listed in the United Network for Organ Sharing (UNOS) database who underwent lung transplantation between May 5, 2005, and December 31, 2022. We used the Kaplan-Meier method to evaluate cumulative survival rates and logistic regression to assess the risk of post-operative complications.
Compared to IPF patients, those with ILD-NSCTD are generally younger, with a lower proportion of male and white patients. After propensity matching, overall survival rates remained similar between the groups (log-rank, p=0.953). However, ILD-NSCTD was associated with a significantly higher risk of post-operative stroke (adjusted OR 1.75, 95% CI 1.12-2.74, p=0.015) and longer post-operative hospital stays (p<0.001). Subgroup analyses yielded consistent results. Finally, infection was identified as the leading cause of death.
Compared to IPF, patients with ILD-NSCTD have a significantly higher risk of post-operative stroke and extended hospital stays, potentially due to complications inherent to ILD-NSCTD. However, the underlying causes of these outcomes remain unclear. Despite these differences, short-term and long-term survival rates are comparable between the two groups, with consistent findings across various ILD-NSCTD subgroups. Therefore, ILD-NSCTD should not be regarded as a relative contraindication for lung transplantation. Nonetheless, the influence of extra-pulmonary complications in ILD-NSCTD patients requires further investigation.
与非硬皮病结缔组织病相关的间质性肺病（ILD-NSCTD）的进行性恶化与预后不良相关，并常导致呼吸衰竭。肺移植（LTx）是一种可行的治疗选择，但其在ILD-NSCTD中的疗效仍存在争议，尤其是在不同亚型之间。
这项回顾性队列研究纳入了在器官共享联合网络（UNOS）数据库中列出的特发性肺纤维化（IPF）患者（n = 11,610）和ILD-NSCTD患者（n = 610），他们在2005年5月5日至2022年12月31日期间接受了肺移植。我们使用Kaplan-Meier方法评估累积生存率，并使用逻辑回归评估术后并发症的风险。
与IPF患者相比，ILD-NSCTD患者通常更年轻，男性和白人患者的比例更低。倾向匹配后，两组的总体生存率相似（对数秩检验，p = 0.953）。然而，ILD-NSCTD与术后中风风险显著更高（调整后的OR为1.75，95% CI为1.12 - 2.74，p = 0.015）以及术后住院时间更长（p < 0.001）相关。亚组分析得出了一致的结果。最后，感染被确定为主要死因。
与IPF相比，ILD-NSCTD患者术后中风风险和住院时间延长的风险显著更高，这可能是由于ILD-NSCTD固有的并发症所致。然而，这些结果的潜在原因仍不清楚。尽管存在这些差异，但两组的短期和长期生存率相当，在各种ILD-NSCTD亚组中均有一致的发现。因此，ILD-NSCTD不应被视为肺移植的相对禁忌证。尽管如此，ILD-NSCTD患者肺外并发症的影响仍需要进一步研究。
