Université Paris-Sud, Faculté de Médecine, Le Kremlin Bicêtre, France AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Le Kremlin Bicêtre, France UMR_S 999, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France
Université Paris-Sud, Faculté de Médecine, Le Kremlin Bicêtre, France AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Service de Pneumologie, Hôpital de Bicêtre, Le Kremlin Bicêtre, France UMR_S 999, INSERM, Laboratoire d'Excellence (LabEx) en Recherche sur le Médicament et l'Innovation Thérapeutique (LERMIT), Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France.
Eur Respir J. 2014 Dec;44(6):1627-34. doi: 10.1183/09031936.00057914. Epub 2014 Oct 16.
Isolated cases of pulmonary arterial hypertension (PAH) in patients treated with interferon (IFN) α or β have been reported in the literature. The aim of this study was to describe all consecutive cases of PAH patients with a history of IFN exposure identified in the French reference centre for severe pulmonary hypertension between 1998 and 2012. A total of 53 patients with PAH and a history of IFN therapy were identified. 48 patients had been treated with IFNα for chronic hepatitis C. Most of them had portal hypertension (85%) and 56% had HIV co-infection. Five additional patients had been treated with IFNβ for multiple sclerosis. The diagnosis of PAH was made within 3 years after IFN therapy in 66% of patients. Repeated haemodynamic assessment was available in 13 out of 16 patients exposed to IFN after the diagnosis of PAH. Increased pulmonary vascular resistance >20% was observed in 11 out of 13 cases (median 43% increase; IQR 32-67%). In five of these patients, IFN withdrawal resulted in spontaneous haemodynamic improvement. This retrospective analysis suggests that IFN therapy may trigger PAH. However, most of these patients had other risk factors for PAH. A prospective case-control study is necessary to definitively establish a link between IFN exposure and PAH.
已有文献报道干扰素（IFN）α或β治疗的患者中孤立性肺动脉高压（PAH）病例。本研究的目的是描述在 1998 年至 2012 年间法国严重肺动脉高压参考中心发现的所有连续有 IFN 暴露史的 PAH 患者病例。共发现 53 例有 PAH 病史且接受过 IFN 治疗的患者。48 例患者因慢性丙型肝炎接受 IFNα治疗。他们中的大多数人患有门静脉高压（85%），56%合并 HIV 感染。另外 5 例患者因多发性硬化症接受 IFNβ治疗。在接受 IFN 治疗后 3 年内诊断为 PAH 的患者占 66%。在诊断为 PAH 后接受 IFN 治疗的 16 例患者中，有 13 例进行了重复血流动力学评估。在 13 例患者中有 11 例（中位数增加 43%；IQR 32-67%）观察到肺血管阻力增加>20%。在这 5 例患者中，IFN 停药导致了自发的血流动力学改善。这项回顾性分析表明 IFN 治疗可能引发 PAH。然而，这些患者大多存在其他 PAH 的危险因素。需要进行前瞻性病例对照研究来确定 IFN 暴露与 PAH 之间的联系。
