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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:
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:
Participants' data will include:
Scoring systems will be calculated using standardized formulas as follows:
This is a retrospective observational study designed to evaluate and compare the prognostic performance of three widely used scoring systems-Gender-Age-Physiology (GAP) Index, Interstitial Lung Disease-GAP (ILD-GAP) Index, and Composite Physiologic Index (CPI)-in patients diagnosed with interstitial lung diseases (ILDs). The study focuses specifically on three ILD subtypes: Idiopathic Pulmonary Fibrosis (IPF), fibrotic Hypersensitivity Pneumonitis (HP), and non-fibrotic HP.
The primary objective is to determine whether GAP, ILD-GAP, and CPI scores can accurately predict overall survival across these patient groups. A secondary aim is to compare the prognostic performance of the scoring systems to explore whether one model provides superior predictive value in specific ILD subtypes.
The study cohort includes adult patients (≥18 years) who were diagnosed between October 2011 and October 2021 at the University of Health Sciences Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital. A total of 143 patients were identified and included: 45 patients with IPF, 26 with fibrotic HP, and 72 with non-fibrotic HP.
Data collected for each patient include clinical and demographic characteristics, duration of symptoms, high-resolution computed tomography (HRCT) findings, pulmonary function test (PFT) results, bronchoalveolar lavage (BAL) findings, and histopathological diagnoses where available. The final diagnosis in each case was established through multidisciplinary team (MDT) discussions and/or histopathological confirmation.
The scoring systems were calculated using standardized formulas:
GAP Index: based on gender, age, and physiology (FVC % predicted, DLCO % predicted).
ILD-GAP Index: a modification of the GAP model for use in connective tissue disease-associated and other ILDs.
CPI (Composite Physiologic Index): calculated as 91 - (0.65 × %DLCO) - (0.53 × %FVC) + (0.34 × %FEV1).
By applying these validated prognostic models to a real-world ILD cohort, the study aims to provide insights into their relative accuracy, clinical utility, and potential limitations. Findings are expected to contribute to more precise prognostic assessment and risk stratification in patients with fibrotic and non-fibrotic ILDs.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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. |
| |
| 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. |
| |
| 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. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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). |
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Association between clinical parameters and survival across ILD subgroups | Evaluation of the relationship between patient age, gender, presence of comorbidities, and symptom profiles and overall survival in IPF, fibrotic HP, and non-fibrotic HP groups using log-rank tests and Cox regression. | Using data collected between October 2011 and October 2021 |
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Inclusion Criteria:
Exclusion Criteria:
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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.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital | Istanbul | Turkey (Türkiye) |
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| ID | Term |
|---|---|
| D000542 | Alveolitis, Extrinsic Allergic |
| D017563 | Lung Diseases, Interstitial |
| D054990 | Idiopathic Pulmonary Fibrosis |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D012130 | Respiratory Hypersensitivity |
| D006969 | Hypersensitivity, Immediate |
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|
| ILD-GAP Index | Other | In addition to the GAP parameters, the index was calculated to include the ILD subtype as well. |
|
| 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 |
|
| Impact of CPI score increase on mortality risk | Assessment of the association between each 1-unit increase in Composite Physiologic Index (CPI) and the risk of mortality using Cox proportional hazards regression. | Using data collected between October 2011 and October 2021 |
| Effect of GAP stage, gender, and CTD status on survival | Stratified survival analysis to investigate whether GAP stage (I-III), female gender, and absence of connective tissue disease are associated with longer survival, using Kaplan-Meier curves and multivariate Cox models. | Using data collected between October 2011 and October 2021 |
| Comparison of prognostic performance (AUC) of GAP, ILD-GAP, and CPI across ILD subtypes | Receiver Operating Characteristic (ROC) analysis to compare the discriminative ability of GAP, ILD-GAP, and CPI scores in predicting mortality. AUC values were reported for each model across IPF, fibrotic HP, and non-fibrotic HP subgroups. | At baseline scoring and during follow-up (using data collected between October 2011 and October 2021) |
| D006967 | Hypersensitivity |
| D007154 | Immune System Diseases |
| D011658 | Pulmonary Fibrosis |