Studying the Treatment Effect of Pirfenidone in Chronic Lung Allograft Dysfunction (STOP-CLAD)

Exclusion Criteria

• •Acute Rejection (AR) diagnosis by biopsy in the 28 days prior to enrollment
• •Treatment with pulse steroids, Anti-thymocyte Globulin (ATG), extracorporeal photopheresis (ECP), plasmapheresis, or Immunoglobulin therapy aimed at CLAD within the 28 days prior to enrollment
• •If the subject is receiving chronic Azithromycin therapy, the dose must be stable for the 28 days prior to enrollment
• •Presence of active pulmonary infection at the time of enrollment as determined by an investigator in consultation with the treating pulmonologist
• •Diagnosis of bronchial stenosis either a) requiring stenting, or b) thought to be responsible for the spirometric decline by principal investigator
• •Abnormal liver function tests (aspartate aminotransferase (AST) or alanine aminotransferase (ALT) \> 2 x upper limit of normal (ULN), Alkaline phosphatase \> 2.5 x ULN, total bilirubin \> ULN) or known cirrhosis (\>2 times upper limit of normal of AST/ALT/AP)
• •Total white blood cell (WBC) \< 3.0 K/uL
• •Moderate to Severe Renal insufficiency (CrCl \<15 mL/min calculated by the Cockcroft-Gault equation)
• •Use of any medication known to cause significant interactions with pirfenidone (strong CYP1A2 inhibitors such as Fluvoxamine or Enoxacin or inducers)
• •Pregnancy or lactation. Women of child-bearing potential will have a pregnancy test at enrollment and must agree to maintain highly effective contraception with two methods of birth control from the date of consent through the end of the study.
• •Tobacco use within 6 months
• •History of alcohol abuse in the past 1 year as determined by the treating pulmonologist
• •Any condition other than CLAD that will likely result in death in the next 1 year
• •Any condition in the judgement of the principal investigator that would preclude participation in this study
• •EKG with QTc interval \> 500 msec at screening
• •Listed for repeat lung transplantation