progressive massive fibrosis MedDRA version: 20.0 Level: PT Classification code 10040678 Term: Silicosis System Organ Class: 10022117 - Injury, poisoning and procedural complications MedDRA version: 21.1 Level: PT Classification code 10036805 Term: Progressive massive fibrosis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders
Conditions
Interventions
Trade Name: Esbriet
Pharmaceutical Form: Capsule, hard
INN or Proposed INN: PIRFENIDONE
CAS Number: 53179-13-8
Concentration unit: mg milligram(s)
Concentration type: equal
Concentration number: 267-
Sponsors
Fundación para la Gestión de la Investigación Biomédica de Cádiz
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Age over 18 years and under 65. 2. Man with a diagnosis of silicosis in the form of PMF by lung or lymph node biopsy, or by radiological criteria. 3. History of exposure to silica in work with artificial can for at least 5 years. 4. Patients capable of consenting to their participation in the study by providing written informed consent, or, if they are not trained, through a legal repressentative. Are the trial subjects under 18? no Number of subjects for this age range: F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range 18 F.1.3 Elderly (>=65 years) no F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: 1. Participation in another clinical trial in the 6 months prior to the start of participation in this study. 2. Hypersensitivity to any of the components of pirfenidone. 3. Biological or farmacological treatment for any other disease or condition related to silicosis or PMF. 4. Concomitant treatment with a drug that can causes pirfenidone interactions. 5. Active infectious disease 6. Any pathology that may condition the evolution of respiratory diseases, including cancer, HIV, HBV, HCV, liver cirrhosis, liver failure, severe kidney failure or any other that in the opinion of the investigator may interfere with the results of the study. 7. Active smoking 8. Laboratory test abnormalities at screening timepoint - Total bilirrubin >2 ULN - AST/SGOT or ALT/SGPT > 2.5 ULN - Alkaline phosphatase >3.0 ULN - Creatinine clearance <40 mL/min (Cockcroft-Gault) 9. Concomitant treatments that may cause serious digestive events. 10. Digestive surgery or similar procedures that may cause digestive intolerances. 11. Not availability to complete all the trial visits 12. Angiodema
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: 1. To evaluate the efficacy of pirfenidone in reducing pulmonary fibrogenic and inflammatory activity in patients with Progressive Massive Fibrosis (PMF), quantified by cell biomarkers. 2. To evaluate the molecular changes that occur after administration of pirfenidone in pulmonary metabolic activity quatified by PET/TC and the link with inflamatory and fibrogenic biomarkers.;Secondary Objective: 1. To assess changes brought about by pirfenidone in the different cells biomarkers patterns and inflamatory activity resulted by PET/TC with 18-FDG 2. To assess radiological changes in TCAR that occur after administration of pirfenidone and the relation with biomarkers and with 18F FDG acquisition. 3. To assess wheter administration of pirfenidone generates changes on standard funtional respiratory explorations. 4. To assess clinical changes (if any) and safety of pirfenidone after administration to patients with PMF.;Primary end point(s): 1- Cell biomarkers in peripheral blood: - Pro/anti fibrotic and pro/anti inflammatory biomarkers: cytokines (IL-1a, IL-1ß, IL-1RA, IL-2, IL-3, IL-4, IL-5, IL-6, IL-7, IL-8, IL-10, IL-12 (p40), IL-12 (p70), IL-13, IL-15, IL-17, IL-18, IP-10, TGF-ß, TNF-a, IFN-?, MIP-1a, MIP-1ß, MCP-1, PDGF, bFGF, MMP1, -2, -7, -9, -10). Immunological biomarkers: CD45, CD45RO, CD45RA, CD3, CD4, CD8, CD19, CD27, CD56, CD126, CD25, INF-?, IL-4, FoxP3, CD196 y CD161;Timepoint(s) of evaluation of this end point: baseline (day 0), month 3, month 6, month 9, month 12. | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary end point(s): • Cell populations in peripheral blood and the impact of treatment in these populations. • Number of adverse events (AE) and adverse reactions (AR), of serious adverse events (SAE) and serious and unexpected adverse reactions (SUSAR). • Respiratory symptoms (cough, expectoration and dyspnea) and quality of life related to health using the EQ-5D 5L test. • Respiratory function variables, including Vital Capacity Forced, Forced Expiratory Volume in the first second, Volume ratio Forced Expiratory in the first second / Forced Vital Capacity and Capacity for CO diffusion, obtained through standardized respiratory function tests. • Radiological categorization by chest radiology and by High Resolution Computed Tomography, following the International Classification of High-resolution Computed Tomography for Occupational and Environmental Respiratory Diseases (ICOERD) (ICOERD).;Timepoint(s) of evaluation of this end point: baseline (day 0), month 3, month 6, month 9, month 12. | — |
Countries
Spain
Contacts
Public ContactLaura Quintana
Fundación para la Gestión de la Investigación Biomédica de Cádiz
Outcome results
None listed