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Interstitial lung diseases (ILDs) are a heterogeneous group of disorders, which encompass a wide range of conditions. In some patients with fibrosing ILDs, a progressive phenotype similar to that observed in idiopathic pulmonary fibrosis (IPF) may develop during the course of the disease (PF-ILD), including patients with systemic sclerosis (SSc)-related ILD.
The aim of the study is to estimate the incidence and prevalence and to describe the characteristics of patients diagnosed with non-IPF PF-ILD and SSc-ILD, to describe the natural course of disease, and to explore the correlation between mortality and Forced Vital Capacity (FVC) of the patients with non-IPF PF-ILD.
This study will be based on two data sources: the French national medico administrative database (SNDS) and the ILD cohort from the National French center for rare pulmonary diseases in Lyon, France.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| PF-ILD and SSc-ILD patients | PF-ILD and SSc-ILD patients |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| epidemiology, healthcare costs in non-IPF PF-ILD and SSc-ILD | Other | epidemiology, characteristics, healthcare resources and associated costs of patients diagnosed with non-IPF PF-ILD and SSc-ILD |
| Measure | Description | Time Frame |
|---|---|---|
| Incidence of PF-ILD and SSc-ILD patients | The study will describe for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) and Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) patients in France.:
| Between 2010 and 2017 |
| Prevalence of PF-ILD and SSc-ILD patients | The study will describe for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) and Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) patients in France.:
| Between 2010 and 2017 |
| characteristics of PF-ILD and SSc-ILD patients | The study will describe for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) and Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) patients in France.:
| Between 2010 and 2017 |
| healthcare resource use of PF-ILD and SSc-ILD patients | The study will describe for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) and Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) patients in France.:
| Between 2010 and 2017 |
| associated costs of PF-ILD and SSc-ILD patients | The study will describe for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) and Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) patients in France.:
|
| Measure | Description | Time Frame |
|---|---|---|
| mortality for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) in France. | correlation between mortality and FVC for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) in France. | Between 2010 and 2017 |
| Forced Vital Capacity for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) in France. |
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Inclusion Criteria:
Exclusion Criteria:
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Patients hospitalized for SSc-ILD, and PF-ILD (excluding the IPF patients)
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Vincent Cottin, Pr | Contact | 472 35 76 53 | +33 | vincent.cottin@chu-lyon.fr |
| Name | Affiliation | Role |
|---|---|---|
| Vincent Cottin, Pr | Hospices Civils de Lyon | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Cardiologique Louis Pradel | Bron | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 34552943 | Derived | Cottin V, Larrieu S, Boussel L, Si-Mohamed S, Bazin F, Marque S, Massol J, Thivolet-Bejui F, Chalabreysse L, Maucort-Boulch D, Jouneau S, Hachulla E, Chollet J, Nasser M. Epidemiology, Mortality and Healthcare Resource Utilization Associated With Systemic Sclerosis-Associated Interstitial Lung Disease in France. Front Med (Lausanne). 2021 Aug 30;8:699532. doi: 10.3389/fmed.2021.699532. eCollection 2021. | |
| 34030695 |
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| Between 2010 and 2017 |
correlation between mortality and FVC for the non-idiopathic pulmonary fibrosis (IPF) progressive fibrosing interstitial lung (PF-ILD) in France. |
| Between 2010 and 2017 |
| Derived |
| Nasser M, Larrieu S, Boussel L, Si-Mohamed S, Bazin F, Marque S, Massol J, Thivolet-Bejui F, Chalabreysse L, Maucort-Boulch D, Hachulla E, Jouneau S, Le Lay K, Cottin V. Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study). Respir Res. 2021 May 24;22(1):162. doi: 10.1186/s12931-021-01749-1. |
| 32943410 | Derived | Nasser M, Larrieu S, Si-Mohamed S, Ahmad K, Boussel L, Brevet M, Chalabreysse L, Fabre C, Marque S, Revel D, Thivolet-Bejui F, Traclet J, Zeghmar S, Maucort-Boulch D, Cottin V. Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study). Eur Respir J. 2021 Feb 11;57(2):2002718. doi: 10.1183/13993003.02718-2020. Print 2021 Feb. |
| ID | Term |
|---|---|
| D017563 | Lung Diseases, Interstitial |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| ID | Term |
|---|---|
| D017048 | Health Care Costs |
| ID | Term |
|---|---|
| D003365 | Costs and Cost Analysis |
| D004467 | Economics |
| D004472 | Health Care Economics and Organizations |
| D003695 | Delivery of Health Care |
| D017530 | Health Care Quality, Access, and Evaluation |
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