There is one clinical trial.
The purpose of this study is to compare the effect of n-acetylcysteine (NAC) plus standard care with matched placebo plus standard of care in patients diagnosed with idiopathic pulmonary fibrosis (IPF) who have the TOLLIP rs3750920 TT genotype. The study will compare the time to a composite endpoint of relative decline in lung function [10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplantation, or all-cause mortality] The secondary objectives will be to examine the effect of NAC on the components of the primary composite endpoint, the rates of clinical events, change in physiology, change in health status, and change in respiratory symptoms.
Inclusion Criteria: - ≥ 40 years of age - Diagnosed with IPF according to 2018 ATS/ERS/JRS/ALAT, confirmed by enrolling investigator - Signed informed consent - If taking pirfenidone or nintedanib, must be on stable dose for at least 6 weeks prior to enrollment visit - Confirmed rs3570920 TT TOLLIP genotype Exclusion Criteria: - Pregnancy or planning to become pregnant - Women of childbearing potential not willing to remain abstinent (refrain from heterosexual intercourse) or use two adequate methods of contraception, including at least one method with a failure rate of <1% per year during study participation - Significant medical, surgical or psychiatric illness that in the opinion of the investigator would affect subject safety, including liver and renal failure - Receipt of an investigational drug or biological agent within the previous 4 weeks of the screening visit or 5 times the half-life, if longer - Supplemental or prescribed NAC therapy within 60 days of enrollment - Listed for lung transplantation at the time of screening - History of lung cancer Inclusion Criteria: - ≥ 40 years of age - Diagnosed with IPF according to 2018 ATS/ERS/JRS/ALAT, confirmed by enrolling investigator - Signed informed consent - If taking pirfenidone or nintedanib, must be on stable dose for at least 6 weeks prior to enrollment visit - Confirmed rs3570920 TT TOLLIP genotype Exclusion Criteria: - Pregnancy or planning to become pregnant - Women of childbearing potential not willing to remain abstinent (refrain from heterosexual intercourse) or use two adequate methods of contraception, including at least one method with a failure rate of <1% per year during study participation - Significant medical, surgical or psychiatric illness that in the opinion of the investigator would affect subject safety, including liver and renal failure - Receipt of an investigational drug or biological agent within the previous 4 weeks of the screening visit or 5 times the half-life, if longer - Supplemental or prescribed NAC therapy within 60 days of enrollment - Listed for lung transplantation at the time of screening - History of lung cancer Idiopathic Pulmonary Fibrosis Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis Fibrosis This is a multi-center, randomized, double-blind, placebo-controlled trial of NAC or placebo in about 200 participants with IPF with a TOLLIP rs3750920 TT genotype.
Description: This is a composite endpoint of time to 10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplant or death from any cause. Respiratory hospitalizations will be determined by a blinded clinical events classification (adjudication) committee.
Measure: Time to one of the following composite endpoint criteria: 10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplant or death from any cause. Time: 24 monthsDescription: This is a composite endpoint of time to 10% relative decline in FVC % predicted, first respiratory hospitalization, lung transplant or death from any cause. Respiratory hospitalizations will be determined by a blinded clinical events classification (adjudication) committee.
Measure: Time to one of the following composite criteria: 10% relative decline in FVC % predicted, first respiratory hospitalization, lung transplant or death from any cause. Time: 24 monthsDescription: Respiratory hospitalizations will be determined by a blinded clinical events classification (adjudication) committee.
Measure: Time to first respiratory hospitalization Time: 24 months