open-label study to assess the effectiveness of pirfenidone in participants with idiopathic pulmonary fibrosis (ipf).

Conditions: Idiopathic Pulmonary Fibrosis

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

Keywords: pirfenidone, uip, ipf, fvc