Golchin Negar, Patel Aditya, Scheuring Julia, Wan Victoria, Hofer Kimberly, Collet Jean-Paul, Elpers Brandon, Lesperance Tamara
Bristol Myers Squibb, Lawrenceville, NJ, USA.
Evidinno Outcomes Research Inc, Vancouver, BC, Canada.
BMC Pulm Med. 2025 Aug 7;25(1):378. doi: 10.1186/s12890-025-03836-1.
Idiopathic pulmonary fibrosis (IPF) is a progressive and serious lung disease with a poor prognosis and severe clinical and humanistic burden. This systematic literature review and meta-analysis aimed to summarize and quantify the data on IPF incidence and prevalence among adults within the general population and to compare regional differences.
Comprehensive searches of MEDLINE, Embase, and the Cochrane Database of Systematic Reviews were conducted to capture available studies published in English from January 1, 2000, to November 7, 2023, that reported on the incidence or prevalence of IPF. Pooled weighted-mean incidence and prevalence estimates were calculated from studies reporting adequate epidemiological data using a DerSimonian-and-Laird random-effects model.
Of 4,077 records identified, 26 studies were included in the meta-analysis (17 reported both prevalence and incidence, 6 reported incidence only, 3 reported prevalence only). Most studies were retrospective, with study periods ranging from 1984 to 2021. Pooled global incidence per 100,000 (95% confidence interval) was 5.8 (4.8, 6.8; 23 studies). Pooled incidence in Asia was 4.4 (1.6, 7.2; 5 studies), 5.1 (3.9, 6.3; 13 studies) in Europe, and 9.0 (6.9, 11.1; 5 studies) in North America. Pooled prevalence (per 100,000) was 17.7 (14.0, 21.5; 20 studies) globally, 14.8 (7.1, 22.6; 6 studies) in Asia, 14.6 (9.4, 19.7; 9 studies) in Europe, and 27.2 (21.0, 33.4; 6 studies) in North America.
This analysis confirms that IPF is a rare condition globally, but substantial heterogeneity exists across studies. Incidence and prevalence were notably high in North America compared with Europe and Asia. This finding may be explained by the use of selective source populations in North American studies, in contrast to the more general populations used in European or Asian studies. Additional contributing factors include variations in case identification algorithms, differences in diagnostic definitions and regional differences in occupational and environmental exposures. While recent multi-societal guidelines have advanced the standardization of the IPF diagnostic process, variability in clinical practice remains a challenge that affects comparisons of incidence and prevalence across regions and over time.
特发性肺纤维化（IPF）是一种进行性严重肺部疾病，预后不良，临床和人文负担沉重。本系统文献综述和荟萃分析旨在总结和量化普通人群中成人IPF发病率和患病率的数据，并比较区域差异。
对MEDLINE、Embase和Cochrane系统评价数据库进行全面检索，以获取2000年1月1日至2023年11月7日以英文发表的报告IPF发病率或患病率的现有研究。使用DerSimonian-Laird随机效应模型，从报告了充分流行病学数据的研究中计算合并加权平均发病率和患病率估计值。
在识别出的4077条记录中，26项研究纳入了荟萃分析（17项报告了患病率和发病率，6项仅报告了发病率，3项仅报告了患病率）。大多数研究为回顾性研究，研究期从1984年至2021年。每10万人的合并全球发病率（95%置信区间）为5.8（4.8，6.8；23项研究）。亚洲的合并发病率为4.4（1.6，7.2；5项研究），欧洲为5.1（3.9，6.3；13项研究），北美为9.0（6.9，11.1；5项研究）。全球合并患病率（每10万人）为17.7（14.0，21.5；20项研究），亚洲为14.8（7.1，22.6；6项研究），欧洲为14.6（9.4，19.7；9项研究），北美为27.2（21.0，33.4；6项研究）。
该分析证实IPF在全球范围内是一种罕见疾病，但各研究之间存在显著异质性。与欧洲和亚洲相比，北美地区的发病率和患病率明显更高。这一发现可能是由于北美研究中使用了选择性来源人群，而欧洲或亚洲研究中使用的是更具普遍性的人群。其他促成因素包括病例识别算法的差异、诊断定义的不同以及职业和环境暴露的区域差异。虽然近期的多社会指南推进了IPF诊断过程的标准化，但临床实践中的变异性仍然是一个挑战，影响着不同地区和不同时间发病率和患病率的比较。
