Costabel Ulrich, Bendstrup Elisabeth, Cottin Vincent, Dewint Pieter, Egan Jim J J, Ferguson James, Groves Richard, Hellström Per M, Kreuter Michael, Maher Toby M, Molina-Molina Maria, Nordlind Klas, Sarafidis Alexandre, Vancheri Carlo
Department of Pneumology/Allergology, Ruhrlandklinik, University Hospital, University Duisburg-Essen, Essen, Germany,
Adv Ther. 2014 Apr;31(4):375-91. doi: 10.1007/s12325-014-0112-1. Epub 2014 Mar 18.
Pirfenidone is currently the only approved therapy for idiopathic pulmonary fibrosis, following studies demonstrating that treatment reduces the decline in lung function and improves progression-free survival. Although generally well tolerated, a minority of patients discontinue therapy due to gastrointestinal and skin-related adverse events (AEs). This review summarizes recommendations based on existing guidelines, research evidence, and consensus opinions of expert authors, with the aim of providing practicing physicians with the specific clinical information needed to educate the patient and better manage pirfenidone-related AEs with continued pirfenidone treatment. The main recommendations to help prevent and/or mitigate gastrointestinal and skin-related AEs include taking pirfenidone during (or after) a meal, avoiding sun exposure, wearing protective clothing, and applying a broad-spectrum sunscreen with high ultraviolet (UV) A and UVB protection. These measures can help optimize AE management, which is key to maintaining patients on an optimal treatment dose.
吡非尼酮是目前唯一被批准用于治疗特发性肺纤维化的药物，此前的研究表明，该治疗可减少肺功能下降并改善无进展生存期。尽管吡非尼酮通常耐受性良好，但少数患者因胃肠道和皮肤相关不良事件（AE）而停止治疗。本综述总结了基于现有指南、研究证据和专家作者共识意见的建议，旨在为执业医师提供教育患者所需的具体临床信息，并在继续使用吡非尼酮治疗时更好地管理与吡非尼酮相关的AE。有助于预防和/或减轻胃肠道和皮肤相关AE的主要建议包括在进餐期间（或之后）服用吡非尼酮、避免阳光照射、穿着防护服以及涂抹具有高紫外线（UV）A和UVB防护功能的广谱防晒霜。这些措施有助于优化AE管理，这是使患者维持最佳治疗剂量的关键。
