Traunero Arianna, Peri Francesca, Badina Laura, Amaddeo Alessandro, Zuliani Elettra, Maschio Massimo, Barbi Egidio, Ghirardo Sergio
Department of Medical, Surgical and Health Sciences, University of Trieste, 34126 Trieste, Italy.
Department of Pediatrics, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, 34137 Trieste, Italy.
Children (Basel). 2023 Sep 11;10(9):1535. doi: 10.3390/children10091535.
Recipients of HSCT have a high risk of infective and non-infective pulmonary diseases. Most patients with pulmonary involvement present multiple pathogenetic mechanisms simultaneously with complex interactions. Therefore, it can be difficult to distinguish the contributions of each one and to perform studies on this subject. In this opinion article, we discuss only chronic pulmonary manifestations, focusing on LONIPCs (late-onset non-infectious pulmonary complications). This term embraces drug-related toxicity, allergies, and chronic pulmonary graft versus host disease (GvHD) in all its recently identified clinical variants. Among LONIPCs, GvHD represents the most critical in terms of morbidity and mortality, despite the rapid development of new treatment options. A recently emerging perspective suggests that pulmonary lung rejection in transplant patients shares striking similarities with the pathogenesis of GvHD. In a pulmonary transplant, the donor organ is damaged by the host immune system, whereas in GvHD, the donor immune system damages the host organs. It constitutes the most significant breakthrough in recent years and is highly promising for both hematologists and thoracic transplant surgeons. The number of patients with LONIPCs is scarce, with heterogenous clinical characteristics often involving several pathogenetic mechanisms, making it challenging to conduct randomized controlled trials. Therefore, the body of evidence in this field is scarce and generally of low quality, leading to jeopardized choices in terms of immunosuppressive treatment. Moreover, it risks being outdated by common practice due to the quick evolution of knowledge about the diagnosis and treatment of LONIPCs. The literature is even more pitiful for children with pulmonary involvement related to HSCT.
造血干细胞移植(HSCT)受者发生感染性和非感染性肺部疾病的风险很高。大多数肺部受累患者同时存在多种致病机制,相互作用复杂。因此,很难区分每种机制的作用并开展相关研究。在这篇观点文章中,我们仅讨论慢性肺部表现,重点关注迟发性非感染性肺部并发症(LONIPCs)。该术语涵盖药物相关毒性、过敏以及慢性移植物抗宿主病(GvHD)的所有最新发现的临床变异类型。在LONIPCs中,尽管新的治疗选择迅速发展,但GvHD在发病率和死亡率方面最为关键。最近出现的一种观点认为,移植患者的肺排斥反应与GvHD的发病机制有惊人的相似之处。在肺移植中,供体器官受到宿主免疫系统的损害,而在GvHD中,供体免疫系统损害宿主器官。这是近年来最重大的突破,对血液科医生和胸外科移植医生都极具前景。LONIPCs患者数量稀少,临床特征各异,常涉及多种致病机制,因此开展随机对照试验具有挑战性。因此,该领域的证据匮乏且质量普遍较低,导致免疫抑制治疗的选择受到影响。此外,由于关于LONIPCs诊断和治疗的知识快速发展,其风险可能因常规做法而过时。对于与HSCT相关的肺部受累儿童,相关文献更为匮乏。