Prognostic Score Comparison in IPF and HP

NCT07169253 | Completed

• 1 other identifier: ILDPrognostic-Scores
• Study Type: observational
• Target: 143
• Locations: 1 country
• Sites: 1

Brief Summary

The goal of this retrospective observational study is to evaluate and compare the prognostic value of GAP, ILD-GAP, and CPI scoring systems in patients diagnosed with interstitial lung diseases (ILDs), specifically Idiopathic Pulmonary Fibrosis (IPF), fibrotic Hypersensitivity Pneumonitis, and non-fibrotic Hypersensitivity Pneumonitis. The main questions it aims to answer are:

• Can GAP, ILD-GAP, and CPI scores accurately predict prognosis in IPF, fibrotic HP, and non-fibrotic HP patients?
• Are there differences in prognostic performance of these models across different ILD subtypes? Researchers will compare the prognostic utility of GAP, ILD-GAP, and CPI scores between IPF, fibrotic HP, and non-fibrotic HP groups to see if one scoring system offers superior predictive power. Participants:
• Adults aged ≥18 years
• Diagnosed between October 2011 and October 2021 at the University of Health Sciences Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital
• 143 patients in total (IPF: 45, fibrotic HP: 26, non-fibrotic HP: 72) Participants' data will include:
• Clinical and demographic characteristics
• Symptom duration
• Radiologic imaging findings
• Pulmonary function test (PFT) results
• Bronchoalveolar lavage (BAL) findings
• Histopathological diagnoses Scoring systems will be calculated using standardized formulas as follows:
• GAP (Gender, Age, Physiology)
• ILD-GAP (modified GAP for CTD-ILDs)
• CPI (Composite Physiologic Index = 91 - \[0.65 × %DLCO\] - \[0.53 × %FVC\] + \[0.34 × %FEV1\])

Conditions

IPF; Fibrotic Lung Disease; Hypersensitivity Pneumonitis; Interstitial Lung Disease (ILD)

Keywords

Idiopathic Pulmonary Fibrosis; Hypersensitivity Pneumonitis; GAP; ILD-GAP; CPI

Outcome Measures

Primary Outcomes (1)

• Prognostic performance of the Gender-Age-Physiology (GAP) Index, Interstitial Lung Disease-GAP (ILD-GAP) Index, and Composite Physiologic Index (CPI) in predicting overall survival

  Prognostic performance of GAP, ILD-GAP, and CPI scoring systems in predicting overall survival among patients with Idiopathic Pulmonary Fibrosis (IPF), fibrotic Hypersensitivity Pneumonitis, and non-fibrotic Hypersensitivity Pneumonitis.

  Using data collected between October 2011 and October 2021

Secondary Outcomes (4)

• Association between clinical parameters and survival across ILD subgroups

  Using data collected between October 2011 and October 2021

• Impact of CPI score increase on mortality risk

  Using data collected between October 2011 and October 2021

• Effect of GAP stage, gender, and CTD status on survival

  Using data collected between October 2011 and October 2021

• Comparison of prognostic performance (AUC) of GAP, ILD-GAP, and CPI across ILD subtypes

  At baseline scoring and during follow-up (using data collected between October 2011 and October 2021)

Study Arms (3)

IPF

The demographic data, symptom duration, clinical and laboratory findings, radiological images, BAL results, and histopathological diagnoses of patients diagnosed with IPF were evaluated according to the 2018 ATS/ERS/JRS/ALAT Clinical Practice Guideline for the Diagnosis of IPF and included in the IPF group.

Other: GAP Index; Other: ILD-GAP Index; Other: Composite Physiologic Index (CPI)

Fibrotic HP

Patients diagnosed with fibrotic HP were classified according to the 2020 ATS/JRS/ALAT Clinical Practice Guideline for the Diagnosis of Hypersensitivity Pneumonitis in Adults.

Other: GAP Index; Other: ILD-GAP Index; Other: Composite Physiologic Index (CPI)

Non-fibrotic HP

Patients diagnosed with non-fibrotic HP were classified according to the 2020 ATS/JRS/ALAT Clinical Practice Guideline for the Diagnosis of Hypersensitivity Pneumonitis in Adults.

Other: GAP Index; Other: ILD-GAP Index; Other: Composite Physiologic Index (CPI)

Interventions

GAP Index OTHER

For each patient in the IPF, fibrotic HP, and non-fibrotic HP groups, the GAP index was calculated based on age, gender, FVC, and DLCO values. According to the GAP index, patients were classified as Stage 1 (0-3 points), Stage 2 (4-5 points), and Stage 3 (6-8 points).

Fibrotic HP; IPF; Non-fibrotic HP

ILD-GAP Index OTHER

In addition to the GAP parameters, the index was calculated to include the ILD subtype as well.

Fibrotic HP; IPF; Non-fibrotic HP

Composite Physiologic Index (CPI) OTHER

The CPI (Composite Physiologic Index) was calculated using the following formula: CPI = 91 - (0.65 × %DLCO) - (0.53 × %FVC) + (0.34 × %FEV1). DLCO: Diffusing Capacity of the Lung for Carbon Monoxide FVC: Forced Vital Capacity FEV₁: Forced Expiratory Volume in One Second

Fibrotic HP; IPF; Non-fibrotic HP

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients diagnosed with Chronic Hypersensitivity Pneumonitis (fibrotic/non-fibrotic) and Idiopathic Pulmonary Fibrosis (IPF) by multidisciplinary discussion between October 2011 and October 2021 at the University of Health Sciences Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital.

You may qualify if:

• patients diagnosed with IPF based on clinical features, laboratory findings, thoracic HRCT results, and/or histopathological confirmation, as determined by a multidisciplinary team discussion.
• patients diagnosed with Fibrotic Hypersensitivity Pneumonitis based on clinical features, laboratory findings, and thoracic HRCT results, confirmed in a multidisciplinary team discussion.
• patients diagnosed with Non-Fibrotic Hypersensitivity Pneumonitis based on clinical features, laboratory findings, and thoracic HRCT results, confirmed in a multidisciplinary team discussion.
• Male and female patients over 18 years of age.

You may not qualify if:

• Patients under 18 years of age

Sponsors & Collaborators

• Saglik Bilimleri Universitesi: lead

Study Sites (1)

Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital

Istanbul, Turkey (Türkiye)

Location

MeSH Terms

Conditions

Alveolitis, Extrinsic Allergic; Lung Diseases, Interstitial; Idiopathic Pulmonary Fibrosis

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Respiratory Hypersensitivity; Hypersensitivity, Immediate; Hypersensitivity; Immune System Diseases; Pulmonary Fibrosis

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Prof. Dr.

Study Record Dates

• First Submitted: August 26, 2025
• First Posted: September 11, 2025
• Study Start: October 1, 2022
• Primary Completion: February 1, 2023
• Study Completion: March 1, 2023
• Last Updated: September 11, 2025

Record last verified: 2025-08

Locations

TU (1)