Construction and validation of the prediction model for survival prognosis of idiopathic pulmonary fibrosis

YANG Shuang; JIANG Hongxia; ZHU Minghui; CHENG Zhenshun

doi:10.14188/j.1671-8852.2026.0806

您当前的位置：

首页 >

文章列表页 >

Construction and validation of the prediction model for survival prognosis of idiopathic pulmonary fibrosis

更新时间：2026-11-05

- Construction and validation of the prediction model for survival prognosis of idiopathic pulmonary fibrosis
- Medical Journal of Wuhan University Vol. 47, Issue 10, Pages: 1372-1381(2026)
- 作者机构：
  
  武汉大学中南医院呼吸与危重症医学科湖北武汉 430071
- 作者简介：
- 基金信息：
- DOI：10.14188/j.1671-8852.2026.0806
  CLC： R563.9
- Received：16 May 2025，
  
  Published：25 October 2026
- 稿件说明：
移动端阅览
杨双,江红霞,朱明辉等.特发性肺纤维化生存预后预测模型的建立及验证[J].武汉大学学报（医学版）,2026,47(10):1372-1381.

YANG Shuang,JIANG Hongxia,ZHU Minghui,et al.Construction and validation of the prediction model for survival prognosis of idiopathic pulmonary fibrosis[J].Medical Journal of Wuhan University,2026,47(10):1372-1381.
杨双,江红霞,朱明辉等.特发性肺纤维化生存预后预测模型的建立及验证[J].武汉大学学报（医学版）,2026,47(10):1372-1381. DOI： 10.14188/j.1671-8852.2026.0806.

YANG Shuang,JIANG Hongxia,ZHU Minghui,et al.Construction and validation of the prediction model for survival prognosis of idiopathic pulmonary fibrosis[J].Medical Journal of Wuhan University,2026,47(10):1372-1381. DOI： 10.14188/j.1671-8852.2026.0806.

摘要

目的

探讨多种血液学和生化指标对特发性肺纤维化（IPF）总体生存期（OS）的预测价值，构建列线图模型以实现个体化预后分层。

方法

回顾性队列研究纳入2014年1月至2024年8月武汉大学中南医院呼吸与危重症医学科收治的确诊IPF患者894例，收集相关指标。最终纳入研究297例，随机分为训练组（208例）及验证组（89例），训练组进行Cox回归结合临床意义及共线性处理构建列线图模型并验证其预测能力。

结果

构建IPF患者列线图模型，进行时间依赖性受试者工作特征（ROC）曲线分析，结果显示，在训练组中，1年、3年、5年OS的曲线下面积（

AUC）

为0.824、0.706、0.666；验证组中，由于样本量限制，1年OS的

AUC

无法可靠估算，3年、5年OS的

AUC

为0.652、0.596。提示短期生存预测能力较强。显示列线图C指数均高于性别⁃年龄⁃生理变量（GAP）模型（训练组中，列线图模型及GAP模型的C指数分别为0.653、0.564；在验证组中，列线图模型及GAP模型的C指数分别为0.593、0.563），提示列线图模型预测能力优于GAP模型。

结论

构建的列线图模型可实现IPF患者OS的个体化风险评估。该模型与传统GAP评分系统相比更优，有望用于临床风险分层与随访决策，具有一定应用价值。

Abstract

Objective

To explore the predictive value of multiple hematological and biochemical indicators for overall survival in patients with idiopathic pulmonary fibrosis (IPF)

and to establish a nomogram model to achieve individualized prognostic stratification.

Methods

This retrospective cohort study enrolled 894 patients diagnosed with IPF admitted to the Department of Respiratory and Critical Care Medicine

Zhongnan Hospital of Wuhan University from January 2014 to August 2024

and collected relevant clinical indicators. A total of 297 patients were finally included in the analysis. The enrolled subjects were randomly divided into a training cohort (208 patients

70%) and a validation cohort (89 patients

30%). Based on the training cohort

Cox regression analysis

combined with clinical significance evaluation and collinearity assessment

was performed to construct a nomogram model

whose predictive performance was subsequently validated.

Results

A nomogram model for patients with IPF was constructed

and time⁃dependent receiver operating characteristic (ROC) curve analysis was performed. The results showed that the area under the curve (

AUC

) values for 1⁃

3⁃

and 5⁃year overall survival in the training cohort were 0.824

0.706

and 0.666

respectively. Due to the limited sample size of the validation cohort

the AUC of 1⁃year OS could not be reliably estimated; the AUC values for 3⁃ and 5⁃year OS were 0.652 and 0.596

respectively. The C⁃index of the nomogram was higher than that of the Gender⁃Age⁃Physiology (GAP) model. In the training cohort

the C⁃indexes of the nomogram model and GAP model were 0.653 and 0.564

respectively. In the validation cohort

the C⁃indexes of the nomogram model and GAP model were 0.593 and 0.563

respectively

demonstrating that the nomogram model possessed better predictive ability compared with the GAP model.

Conclusion

The established nomogram model enables individualized risk assessment of overall survival in IPF patients. Compared with the traditional GAP scoring system

this model has better predictive performance

which is expected to be applied in clinical risk stratification and follow‑up decision‑making

and has potential clinical value.

关键词

Keywords

references

KING T E , PARDO A , SELMAN M . Idiopathic pulmonary fibrosis [J ] . Lancet , 2011 , 378 ( 9807 ): 1949 ‑ 1961 .

RICHELDI L , COLLARD H R , JONES M G . Idiopathic pulmonary fibrosis [J ] . Lancet , 2017 , 389 ( 10082 ): 1941 ‑ 1952 .

KOUDSTAAL T , WIJSENBEEK M S . Idiopathic pulmonary fibrosis [J ] . La Presse Médicale , 2023 , 52 ( 3 ): 104166 .

LEDERER D J , MARTINEZ F J . Idiopathic pulmonary fibrosis [J ] . N Engl J Med , 2018 , 378 ( 19 ): 1811 ‑ 1823 .

NATSUIZAKA M , CHIBA H , KURONUMA K , et al . Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences [J ] . Am J Respir Crit Care Med , 2014 , 190 ( 7 ): 773 ‑ 779 .

LEY B , RYERSON C J , VITTINGHOFF E , et al . A multidimensional index and staging system for idiopathic pulmonary fibrosis [J ] . Ann Intern Med , 2012 , 156 ( 10 ): 684 ‑ 691 .

SCOTT M K D , QUINN K , LI Q , et al . Increased monocyte count as a cellular biomarker for poor outcomes in fibrotic diseases: A retrospective, multicentre cohort study [J ] . Lancet Respir Med , 2019 , 7 ( 6 ): 497 ‑ 508 .

XUE J M , KASS D J , BON J , et al . Plasma B lymphocyte stimulator and B cell differentiation in idiopathic pulmonary fibrosis patients [J ] . J Immunol , 2013 , 191 ( 5 ): 2089 ‑ 2095 .

ZIEGENHAGEN M W , ZABEL P , ZISSEL G , et al . Serum level of interleukin 8 is elevated in idiopathic pulmonary fibrosis and indicates disease activity [J ] . Am J Respir Crit Care Med , 1998 , 157 ( 3 Pt 1 ): 762 ‑ 768 .

KREUTER M , LEE J S , TZOUVELEKIS A , et al . Monocyte count as a prognostic biomarker in patients with idiopathic pulmonary fibrosis [J ] . Am J Respir Crit Care Med , 2021 , 204 ( 1 ): 74 ‑ 81 .

TEOH A K Y , JO H E , CHAMBERS D C , et al . Blood monocyte counts as a potential prognostic marker for idiopathic pulmonary fibrosis: Analysis from the Australian IPF registry [J ] . Eur Respir J , 2020 , 55 ( 4 ): 1901855 .

HIRATA M , HARA Y , FUJII H , et al . ILD‑GAP combined with the monocyte ratio could be a better prognostic prediction model than ILD‑GAP in patients with interstitial lung diseases [J ] . BMC Pulm Med , 2024 , 24 ( 1 ): 16 .

CHENG X Y , FENG Z C , PAN B Y , et al . Establishment and application of the BRP prognosis model for idiopathic pulmonary fibrosis [J ] . J Transl Med , 2023 , 21 ( 1 ): 805 .

ZINELLU A , COLLU C , NASSER M , et al . The aggregate index of systemic inflammation (AISI): A novel prognostic biomarker in idiopathic pulmonary fibrosis [J ] . J Clin Med , 2021 , 10 ( 18 ): 4134 .

CHEN R X , DAI J H . Lipid metabolism in idiopathic pulmonary fibrosis: From pathogenesis to therapy [J ] . J Mol Med , 2023 , 101 ( 8 ): 905 ‑ 915 .

RAGHU G , REMY‑JARDIN M , MYERS J L , et al . Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline [J ] . Am J Respir Crit Care Med , 2018 , 198 ( 5 ): e44 ‑ e68 .

RAGHU G , COLLARD H R , EGAN J J , et al . An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence‑based guidelines for diagnosis and management [J ] . Am J Respir Crit Care Med , 2011 , 183 ( 6 ): 788 ‑ 824 .

RAGHU G , REMY‑JARDIN M , RICHELDI L , et al . Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: An official ATS/ERS/JRS/ALAT clinical practice guideline [J ] . Am J Respir Crit Care Med , 2022 , 205 ( 9 ): e18 ‑ e47 .

HEUKELS P , MOOR C C , VON DER THÜSEN J H , et al . Inflammation and immunity in IPF pathogenesis and treatment [J ] . Respir Med , 2019 , 147 : 79 ‑ 91 .

OBAYASHI Y , YAMADORI I , FUJITA J , et al . The role of neutrophils in the pathogenesis of idiopathic pulmonary fibrosis [J ] . Chest , 1997 , 112 ( 5 ): 1338 ‑ 1343 .

KARAMPITSAKOS T , TORRISI S , ANTONIOU K , et al . Increased monocyte count and red cell distribution width as prognostic biomarkers in patients with Idiopathic Pulmonary Fibrosis [J ] . Respir Res , 2021 , 22 ( 1 ): 140 .

GREGORY A D , KLIMENT C R , METZ H E , et al . Neutrophil elastase promotes myofibroblast differentiation in lung fibrosis [J ] . J Leukoc Biol , 2015 , 98 ( 2 ): 143 ‑ 152 .

ZINELLU A , PALIOGIANNIS P , SOTGIU E , et al . Blood cell count derived inflammation indexes in patients with idiopathic pulmonary fibrosis [J ] . Lung , 2020 , 198 ( 5 ): 821 ‑ 827 .

ADEGUNSOYE A , HRUSCH C L , BONHAM C A , et al . Skewed lung CCR4 to CCR6 CD4 + T cell ratio in idiopathic pulmonary fibrosis is associated with pulmonary function [J ] . Front Immunol , 2016 , 7 : 516 .

HACHISU Y , MURATA K , TAKEI K , et al . Possible serological markers to predict mortality in acute exacerbation of idiopathic pulmonary fibrosis [J ] . Medicina , 2019 , 55 ( 5 ): 132 .

ILLINGWORTH D R . Lipoprotein metabolism [J ] . Am J Kidney Dis , 1993 , 22 ( 1 ): 90 ‑ 97 .

KOVANEN P T . Regulation of plasma cholesterol by hepatic low‑density lipoprotein receptors [J ] . Am Heart J , 1987 , 113 ( 2 ): 464 ‑ 469 .

SHI X G , CHEN Y H , LIU Q M , et al . LDLR dysfunction induces LDL accumulation and promotes pulmonary fibrosis [J ] . Clin Transl Med , 2022 , 12 ( 1 ): e711 .

Views

下载量

CSCD

Alert me when the article has been cited

提交

Tools

Publicity Resources

Construction of a prognostic nomogram model for sepsis patients based on novel composite indicators

Development and validation of a prognosis⁃related model for female lung cancer based on GEO database

Values of prognostic nutritional index in predicting the outcomes of COVID⁃19 patients

Biomechanical injury induces mitochondrial autophagy in L929 cells by downregulating the TGF‐β/Smad3 signaling pathway

Preparation of methylene blue‐loaded nanoparticles and a preliminary study of photodynamic anti‐Trichophyton rubrum effect in vitro

Related Author

LUO Yan

ZHAN Liying

HUANG Jianhui

WANG Junqiang

WANG Juan

OUYANG Jin

LI Shujun

SUN Chengcao

Related Institution

Intensive Care Unit, Luzhou Traditional Chinese Medicine Hospital

Intensive Care Unit, Renmin Hospital of Wuhan University

School of Public Health, Wuhan University

School of Public Health, Nanchang University

Wuhan Hospital for the Prevention and Treatment of Occupational Diseases

AI问答

⁰