Idiopathic pulmonary fibrosis (IPF) is a rare condition for which reliable disease trends over time can be difficult to obtain. Understanding the epidemiology of IPF is crucial given its impact on quality of life and productivity of affected individuals and their families,1 as well as substantial costs to the healthcare system.2 The occurrence of IPF over time has likely changed with growing awareness of the disease, the advent of antifibrotic therapies that slow disease progression and multiple promising clinical trials that may alter the disease’s natural history. Knowledge of the incidence and prevalence of IPF informs healthcare needs, which range from access to specialists and pharmacotherapy to availability of hospice and resources for end-of-life care. Although smaller studies based on primary sources (eg, medical chart review) exist,3 4 many epidemiological studies on IPF have used patient registries.5 Clinical registries allow for increased accuracy in case identification and more comprehensive data collection,6 which is particularly helpful in complex conditions like pulmonary fibrosis. Studying epidemiology using clinical registries is limited by the costs required to build and maintain them, evolving diagnostic criteria that may affect case identification,7 8 and referral bias given these databases are often located in …

中文翻译：

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特发性肺纤维化的流行病学：罕见疾病人群水平研究的机遇和障碍

特发性肺纤维化 (IPF) 是一种罕见疾病，很难获得可靠的疾病随时间变化的趋势。鉴于 IPF 对受影响个人及其家庭的生活质量和生产力的影响，1 以及医疗保健系统的巨大成本，了解 IPF 的流行病学至关重要。2 随着人们对该疾病认识的不断增强，IPF 的发生率可能会随着时间的推移而发生变化、减缓疾病进展的抗纤维化疗法的出现以及可能改变疾病自然史的多项有希望的临床试验。了解 IPF 的发病率和患病率可以了解医疗保健需求，包括获得专家和药物治疗，以及临终关怀和临终关怀资源的可用性。尽管存在基于主要来源（例如病历审查）的小型研究，3 4 许多关于 IPF 的流行病学研究都使用了患者登记。5 临床登记可以提高病例识别的准确性和更全面的数据收集，6 这对于以下方面特别有帮助：复杂的情况，如肺纤维化。使用临床登记处研究流行病学受到建立和维护它们所需的成本、可能影响病例识别的不断变化的诊断标准的限制，7 8 以及考虑到这些数据库通常位于……的转介偏差。