Pulmonology Department, Coimbra University Hospital, University of Coimbra, Portugal.
Pulmonology Department, São João Hospital Centre, Faculty of Medicine of Porto University, Oporto, Portugal.
Pulmonology. 2021 Jan-Feb;27(1):7-13. doi: 10.1016/j.pulmoe.2020.05.017. Epub 2020 Jun 16.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease of unknown cause, which predominantly manifests in older males. IPF diagnosis is a complex, multi-step process and delay in diagnosis cause a negative impact on patient survival. Additionally, a multidisciplinary team of pulmonologists, radiologists and pathologists is necessary for an accurate IPF diagnosis. The present study aims to assess how diagnosis and treatment of IPF are followed in Portugal, as well as the knowledge and implementation of therapeutic guidelines adopted by the Portuguese Society of Pulmonology.
Seventy-eight practicing pulmonologists were enrolled (May-August 2019) in a survey developed by IPF expert pulmonologists comprised of one round of 31 questions structured in three parts. The first part was related to participant professional profile, the second part assessed participant level of knowledge and practice agreement with national consensus and international guidelines for IPF as well as their access to radiology and pathology for IPF diagnosis, and the third part was a self-evaluation of the guidelines adherence for diagnosis and treatment in their daily practice.
Participants represented a wide spectrum of pulmonologists from 14 districts of Portugal and autonomous regions of Azores and Madeira. The majority were female (65%), with 5-19 years of experience (71%) and working in a public clinical center (83%). Importantly, the majority of pulmonologists follow their IPF patients (n=45) themselves, while 26% referred IPF patients to ILD experts in the same hospital and 22% to another center. Almost all pulmonologists (98%) agreed or absolutely agreed that multidisciplinary discussion is recommended to accurately diagnose IPF. No pulmonologists considered pulmonary biopsy as absolutely required to establish an IPF diagnosis. However, 87% agreed or absolutely agree with considering biopsy in a possible/probable UIP context. If a biopsy is necessary, 96% of pulmonologists absolutely agree or agree with considering criobiopsy as an option for IPF diagnosis. Regarding IPF treatment, 98% absolutely agreed or agreed that antifibrotic therapy should be started once the IPF diagnosis is established. Finally, 76% stated that 6 months is the recommended time for follow-up visit in IPF patients.
Portuguese pulmonologists understand and agree with national consensus and international guidelines for IPF treatment but their implementation in Portugal is heterogeneous.
特发性肺纤维化（IPF）是一种病因不明的慢性、进行性纤维性肺间质疾病，主要发生在老年男性中。IPF 的诊断是一个复杂的、多步骤的过程，诊断的延迟会对患者的生存产生负面影响。此外，需要由肺病学家、放射科医生和病理学家组成的多学科团队来进行准确的 IPF 诊断。本研究旨在评估葡萄牙的 IPF 诊断和治疗情况，以及葡萄牙肺病学会采用的治疗指南的知识和实施情况。
2019 年 5 月至 8 月，对 78 名执业肺病学家进行了一项调查，该调查由 IPF 专家肺病学家制定，共包括一轮 31 个问题，分为三个部分。第一部分与参与者的专业概况有关，第二部分评估了参与者对国家共识和国际 IPF 指南的知识和实践的一致性，以及他们获得放射学和病理学进行 IPF 诊断的情况，第三部分是对他们在日常实践中诊断和治疗指南的自我评估。
参与者代表了葡萄牙 14 个地区和亚速尔群岛和马德拉自治区的广泛的肺病学家。大多数为女性（65%），有 5-19 年的从业经验（71%），在公立医院临床中心工作（83%）。重要的是，大多数肺病学家自己跟踪他们的 IPF 患者（n=45），而 26%的人将 IPF 患者转介到同一家医院的ILD 专家，22%的人转介到另一家中心。几乎所有的肺病学家（98%）都同意或完全同意，建议多学科讨论以准确诊断 IPF。没有肺病学家认为肺活检是确定 IPF 诊断的绝对必要条件。然而，87%的人同意或完全同意在可能/疑似 UIP 情况下考虑活检。如果需要活检，96%的肺病学家完全同意或同意考虑 criobiopsy 作为 IPF 诊断的一种选择。关于 IPF 的治疗，98%的人完全同意或同意一旦确诊 IPF，就应开始使用抗纤维化治疗。最后，76%的人表示，在 IPF 患者中，建议 6 个月进行一次随访。
葡萄牙肺病学家理解并同意 IPF 治疗的国家共识和国际指南，但它们在葡萄牙的实施情况存在差异。
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