Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Respiratory Disease Unit, University of Padova, Padova, Italy.
Department of Medicine, University of British Columbia and Centre for Heart Lung Innovation, St Paul's Hospital, Vancouver, BC, Canada.
Lancet Respir Med. 2021 Sep;9(9):1065-1076. doi: 10.1016/S2213-2600(21)00017-5. Epub 2021 Jul 28.
Many patients with interstitial lung disease (ILD) develop pulmonary fibrosis, which can lead to reduced quality of life and early mortality. Patients with fibrotic ILD often have considerable diagnostic delay, and are exposed to unnecessary and costly diagnostic procedures, and ineffective and potentially harmful treatments. Non-specific and insidious presenting symptoms, along with scarce knowledge of fibrotic ILD among primary care physicians and non-ILD experts, are some of the main causes of diagnostic delay. Here, we outline and discuss the challenges facing both patients and physicians in making an early diagnosis of fibrotic ILD, and explore strategies to facilitate early identification of patients with fibrotic ILD, both in the general population and among individuals at highest risk of developing the disease. Finally, we discuss controversies and key uncertainties in screening programmes for fibrotic ILD. Timely identification and accurate diagnosis of patients with fibrotic ILD poses several substantial clinical challenges, but could potentially improve outcomes through early initiation of appropriate management.
许多间质性肺疾病(ILD)患者会发展为肺纤维化,这会导致生活质量下降和早期死亡。纤维化 ILD 患者通常存在相当大的诊断延迟,并且会接受不必要且昂贵的诊断程序以及无效且可能有害的治疗。非特异性和隐匿性的表现症状,以及初级保健医生和非 ILD 专家对纤维化 ILD 的认识不足,是导致诊断延迟的主要原因之一。在这里,我们概述并讨论了患者和医生在早期诊断纤维化 ILD 方面面临的挑战,并探讨了在普通人群和疾病风险最高的个体中,促进早期识别纤维化 ILD 患者的策略。最后,我们讨论了纤维化 ILD 筛查计划中的争议和关键不确定性。及时识别和准确诊断纤维化 ILD 患者面临着许多重大的临床挑战,但通过早期开始适当的治疗,可能会改善患者的预后。