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
Study Type
OBSERVATIONAL
Enrollment
214
nintedanib
Chang-Hua Christian Hospital
Changhua, Taiwan
Chang Gung Memorial Hospital Chiayi
Chiayi City, Taiwan
National Taiwan University Hospital-Hsin-Chu Branch
Hsinchu, Taiwan
Kaohsiung Medical University Chung-Ho Memorial Hospital
Kaohsiung City, Taiwan
Kaohsiung Veterans General Hospital
Kaohsiung City, Taiwan
E-Da Hospital
Kaohsiung City, Taiwan
Kaohsiung Chang Gung Memorial Hospital
Kaohsiung City, Taiwan
Taipei Tzu Chi General Hospital
New Taipei City, Taiwan
Taipei Medical University-Shuang Ho Hospital
New Taipei City, Taiwan
Chung Shan Medical University Hospital
Taichung, Taiwan
...and 16 more locations
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
Time frame: Up to 5 years
Annual decline from baseline in Diffusing capacity of the Lungs for Carbon monoxide (DLco, %)
Time frame: Up to 5 years
Annual decline from baseline in resting and exercise Oxygen Saturation (SpO2, %)
Time frame: Up to 5 years
Time to first acute exacerbation (AE) of IPF; or time to ILD worsening for SSc-ILD/PF-ILD after study enrollment
Time frame: 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
Time frame: Up to 5 years
Annual change from baseline in Chronic Obstructive Pulmonary Disease (COPD) Assessment Test (CAT)
Time frame: Up to 5 years
Annual change from baseline in Six-Minutes Walking Test (6MWT)
Time frame: Up to 5 years
Annual change from baseline in Berlin questionnaire
Time frame: 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
Time frame: Up to 5 years
Mortality (with cause of death): respiratory- and non-respiratory-related death
Time frame: Up to 5 years
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