NICEFIT-ON: A Study Under Routine Clinical Practice in Taiwan to Observe the Long-term Outcome of People With Certain Types of Lung Disease (PF-ILD, SSc-ILD, IPF) Who Start Treatment With Nintedanib

Study Overview
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Brief Summary
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To better understand the clinical characteristics of Idiopathic Pulmonary Fibrosis (IPF) / Systemic Sclerosis-associated-Interstitial Lung Disease (SSc-ILD)/ Progressive Fibrosing Interstitial Lung Disease (PF-ILD) patients treated with nintedanib and biomarkers associated with the disease course, a non-interventional, 3-year, prospective study will be conducted to collect the long-term real-world clinical data on IPF/SSc-ILD/PF-ILD patients newly administered with nintedanib in Taiwan

Official Title
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Non-Interventional Collecting Evidences For ILD in Taiwan: Optimized Novel Therapy

Conditions
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Idiopathic Pulmonary Fibrosis

Intervention / Treatment
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* Drug: OFEV®


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: This study plans to enroll approximately 500 patients with IPF/SSc-ILD/PF-ILD who newly initiate nintedanib per physicians' discretion within 6 months before participating in the study. IPF cohort: Diagnosed with IPF during the prior 6 months before study enrollment, based on the 2018 ATS/ERS/JRS/ALAT guideline Patient ≥ 40 years of age Newly initiating nintedanib within 6 months prior to participating in the study Providing written informed consent prior to participating in the study Having further follow-up possibility with participating physician during the planned study period Ability to read and write in local language SSc-ILD cohort: Diagnosed with SSc-ILD during the prior 6 months before study enrollment, based on 2013 ACR/EULAR Patient ≥ 20 years of age Newly initiating nintedanib OR not receiving nintedanib per physician's discretion (For patients who diagnosed with SSc-ILD but are not treated with nintedanib on physician's discretion, they will apply the same inclusion criteria, with baseline characteristics collected only) within 6 months prior to participating in the study Providing written informed consent prior to participating in the study Having further follow-up possibility with participating physician during the planned study period Ability to read and write in local language PF-ILD cohort: Diagnosed with PF-ILD (PF-ILD patients will be enrolled only after nintedanib acquires the label approval from TFDA) during the prior 6 months before study enrollment. The definition of PF-ILD diagnosis is as follows: --Patients who have ILD with a progressive phenotype, but are not diagnosed with IPF, per physician's judgment. The pathophysiology in these patients is characterized by self-sustaining fibrosis and a deterioration in lung function over time, with worsening respiratory symptoms, resistance to immune-modulatory therapies, and ultimately early mortality. Patient ≥ 20 years of age Newly initiating nintedanib OR not receiving nintedanib per physician's discretion (For patients who diagnosed with PF-ILD but are not treated with nintedanib on physician's discretion, they will apply the same inclusion criteria, with baseline characteristics collected only) within 6 months prior to participating in the study Providing written informed consent prior to participating in the study Having further follow-up possibility with participating physician during the planned study period Ability to read and write in local language Exclusion Criteria: - Lung transplantation expected within the next 6 months. --Included in ongoing interventional trials

Ages Eligible for Study
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Minimum Age: 20 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Idiopathic Pulmonary Fibrosis (IPF)<br> | Drug: OFEV®<br>* nintedanib<br>|
| Progressive Fibrosing Interstitial Lung Disease (PF-ILD)<br> | Drug: OFEV®<br>* nintedanib<br>|
| Systemic Sclerosis-associated-Interstitial Lung Disease (SSc-ILD)<br> | Drug: OFEV®<br>* nintedanib<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Annual percentage of decline from baseline in Forced Vital Capacity (FVC, %) per cohort of IPF, SSc-ILD, or PF-ILD | IPF: Idiopathic Pulmonary Fibrosis PF-ILD: Progressive Fibrosing Interstitial Lung Disease SSc-ILD: Systemic Sclerosis-associated-Interstitial Lung Disease | Up to 5 years | 
| Annual decline from baseline in Diffusing capacity of the Lungs for Carbon monoxide (DLco, %) |  | Up to 5 years | 
| Annual decline from baseline in resting and exercise Oxygen Saturation (SpO2, %) |  | Up to 5 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Time to first acute exacerbation (AE) of IPF; or time to ILD worsening for SSc-ILD/PF-ILD after study enrollment |  | Up to 5 years | 
| Annual change from baseline in St George's Respiratory Questionnaire (SGRQ) for IPF or King's Brief Interstitial Lung (K-BILD) for other ILDs | IPF: Idiopathic Pulmonary Fibrosis ILD: Interstitial Lung Disease | Up to 5 years | 
| Annual change from baseline in Chronic Obstructive Pulmonary Disease (COPD) Assessment Test (CAT) |  | Up to 5 years | 
| Annual change from baseline in Six-Minutes Walking Test (6MWT) |  | Up to 5 years | 
| Annual change from baseline in Berlin questionnaire |  | Up to 5 years | 
| Change from baseline in quantification of biomarkers | Biomarkers include but not limited to Platelet Derived Growth Factor (PDGF), Vascular Endothelial Growth Factor (VEGF), Fibroblast Growth Factor (FGF), Transforming Growth Factor β1 (TGF-β1), Hepatocyte Growth Factor (HGF), Matrix Metalloproteinase (MMP): MMP-1, MMP-7, MMP-9, α-defensin 1, High Mobility Group Box 1 (HMGB1), Tissue of Metalloproteinase (TIMP), Heat-Shock Protein (HSP): HSP-27, bile acid conjugated, Lysophosphatidic Acid (LPA), Lysophosphatidic Acid Receptor 1 (LPAR1), Prostagladin E2 (PGE2), Interleukin (IL): IL-1β, IL-4, IL-18, IL-13, IL-17, Monocyte Chemoattractant Protein 1 (MCP-1), Macrophage Inflammatory Protein 2 (MIP-2), periostin, osteopontin, Surfactant Protein A (SPA), Surfactant Protein D (SPD), Krebs von den Lungen 6 / Mucin 1 (KL-6/MUC1), anti-HSP70 Immunoglobolin (IgG), Bone Morphogenic Protein (BMP), Carbonhydrate Antigen-199 (CA-199), C-Reaktiv Protein degraded by MMPs (CRPM), chemokine ligand (CCL): CCL 2, CCL-18 | Up to 5 years | 
| Mortality (with cause of death): respiratory- and non-respiratory-related death |  | Up to 5 years |