Prospective Treatment Efficacy in IPF Using Genotype for Nac Selection (PRECISIONS) Trial

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

   - Inability to perform spirometry

   - Forced vital capacity (FVC) less than 45% predicted, using the global lung function
   index (GLI) equation at Visit 1

   - Active respiratory infection requiring treatment with antibiotics within 4 weeks of
   Visit 1