Open-label Study to Assess the Effectiveness of Pirfenidone in Participants With Idiopathic Pulmonary Fibrosis (IPF).
Condition: Idiopathic Pulmonary Fibrosis
MeSH terms: Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis; Fibrosis
Intervention: Pirfenidone
Intervention description: Pirfenidone 2403 mg/d capsules orally will be given in divided doses (TID) after titration period of 14 days.
Intervention MeSH terms: Pirfenidone
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 60.0
Sex: All
Minimum age: 40 Years
Maximum age: 80 Years
Healthy volunteers: No
Keywords: Pirfenidone, UIP, IPF, FVC

Summary:

      This study is a national, multicenter, interventional, non-randomized, non-controlled,
      open-label study to assess the effectiveness of pirfenidone in participants with IPF in
      Russian clinical practice.
    

Eligibility criteria:

        Inclusion Criteria:

          -  Clinical symptoms consistent with IPF of ≥ 6months duration

          -  Participants could have both "confident" or "consistent" with UIP diagnosis of IPF
             based on clinical, radiologic and pathologic data according to 2011 American Thoracic
             Society/European Respiratory Society (ATS/ERS) guidelines at the Screening. HRCT scan
             performed within 24 months before the start of the Screening may be used, if it meets
             all image acquisition guideline

          -  No features supporting an alternative diagnosis on transbronchial biopsy,
             bronchoalveolar lavage (BAL), or surgical lung biopsy, if performed. Results of the
             surgical lung biopsy performed within the last 4 years must be confirmed by central
             review

          -  Participants with %FVC ≥ 40 % at the Screening

          -  Participants with %Carbon monoxide diffusing capacity (DLCO) ≥ 30 % at the Screening

          -  Ability to walk ≥ 100 m during the 6-minute walk test at the Screening

          -  Eligible participants must discontinue all prohibited medications at least 28 days
             before the Screening

          -  Female participants of childbearing potential must have negative urine pregnancy test
             at the Screening and before first dosing on Day 1

        Exclusion Criteria:

          -  Significant clinical worsening of IPF between Screening and Day 1, in the opinion of
             the investigator

          -  Relevant airways obstruction (i.e. pre-bronchodilator forced expiratory volume
             (FEV)1/FVC < 0.7)

          -  Cigarette smoking within 28 days before the start of treatment or unwilling to avoid
             tobacco products throughout the study

          -  History of clinically significant environmental exposure known to cause pulmonary
             fibrosis (PF), including but not limited to drugs (such as amiodarone), asbestos,
             beryllium, radiation, and domestic birds

          -  Known explanation for interstitial lung disease, including but not limited to
             radiation, drug toxicity, sarcoidosis, hypersensitivity pneumonitis, bronchiolitis
             obliterans organizing pneumonia, human immunodeficiency virus (HIV), viral hepatitis,
             and cancer

          -  Clinical diagnosis of any connective tissue disease, including but not limited to
             scleroderma, polymyositis/ dermatomyositis, systemic lupus erythematosus, and
             rheumatoid arthritis

          -  During baseline analysis of HRCT, significant coexistent emphysema (emphysema extent
             greater than extent of fibrosis) confirmed by central review

          -  Planned lung transplantation during the study

          -  Clinical evidence of active infection, including but not limited to bronchitis,
             pneumonia, sinusitis, urinary tract infection, or cellulitis

          -  Unable to perform 6MWT or to undergo pulmonary function test

          -  Any history of malignancy likely to result in significant disability or likely to
             require significant medical or surgical intervention within the next 1 years. This
             does not include minor surgical procedures for localized cancer (e.g., basal cell
             carcinoma)

          -  History of severe hepatic impairment or end-stage liver disease

          -  History of end-stage renal disease requiring dialysis

          -  History of unstable or deteriorating cardiac or pulmonary disease (other than IPF)
             within the previous 6 months

          -  Pregnancy or lactation, or intention to become pregnant during the study. Women of
             childbearing capacity are required to have a negative urine pregnancy test before
             treatment and must agree to maintain highly effective contraception

          -  Liver function test outside specified limits at the Screening: total bilirubin above
             the upper limit of normal (ULN); aspartate or alanine aminotransferase (AST or ALT) >
             3 × ULN; alkaline phosphatase > 2.5 × ULN

          -  Creatinine clearance < 30 mL/min, calculated using the Cockcroft-Gault formula

          -  Electrocardiogram (ECG) with a QT interval corrected according to Fridericia's formula
             (QTcF) > 500 msec at the Screening