Τίτλος:
Safety and efficacy of pirfenidone in severe Idiopathic Pulmonary Fibrosis: A real-world observational study
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Background Pirfenidone is a novel anti-fibrotic drug that has shown efficacy in five randomized multicenter clinical trials enrolling patients with Idiopathic Pulmonary Fibrosis of mild-to-moderate disease severity. Scarce data supports the use of pirfenidone in IPF patients with more advanced disease. Objective To investigate the safety and efficacy profile of pirfenidone in IPF patients with severe lung function impairment. Patients and methods This was a retrospective study enrolling patients with advanced IPF (FVC%predicted < 50% and/or (DLco%predicted <35%) receiving pirfenidone for at least 6 months. Results Between September 2011 and March 2013, we identified 43 patients with severe IPF (baseline meanFVC%predicted±SD: 63.8 ± 20.3, meanDLCO%predicted: 27.3 ± 8.2), of mean age±SD: 66.3 + 9.7, 34 males (81%) that received pirfenidone (2.403 mg/daily) for one year. Pirfenidone treatment was associated with a trend towards decrease in functional decline compared to 6-months before treatment initiation but failed to show any benefit after one year of treatment (ΔFVC: −3.3 ± 4.6 vs 0.49 ± 11.4 and vs. −5.8 ± 11.8, p = 0.06 and p = 0.04, respectively and ΔDLCO: −13.3 ± 15.2 vs. −10.1 ± 16.6 and vs. 28.3 ± 19.2, p = 0.39 and p = 0.002, respectively). Gastrointestinal disorders (34.9%), fatigue (23.2%) and photosensitivity (18.6%) were the most common adverse events. Adverse events led to treatment discontinuation in 9 patients (20.9%) and dose reduction in 14 (32.5%). Conclusion Pirfenidone appears to be safe when administered in patients with advanced IPF. Pirfenidone efficacy in IPF patients with severe lung function impairment may diminish after 6 months of treatment. © 2017 Elsevier Ltd
Συγγραφείς:
Tzouvelekis, A.
Ntolios, P.
Karampitsakos, T.
Tzilas, V.
Anevlavis, S.
Bouros, E.
Steiropoulos, P.
Koulouris, N.
Stratakos, G.
Froudarakis, M.
Bouros, D.
Περιοδικό:
Pulmonary Pharmacology and Therapeutics
Εκδότης:
INSTAP Academic Press
Λέξεις-κλειδιά:
pirfenidone; placebo; nonsteroid antiinflammatory agent; pirfenidone; pyridone derivative, aged; anorexia; Article; clinical article; community acquired pneumonia; controlled study; diarrhea; disease course; disease severity; drug dose reduction; drug efficacy; drug safety; drug withdrawal; fatigue; female; fibrosing alveolitis; forced vital capacity; heart infarction; hematuria; human; insomnia; liver toxicity; lung function; male; multicenter study; nausea and vomiting; observational study; photosensitivity; priority journal; prospective study; randomized controlled trial; retrospective study; septic shock; six minute walk test; total lung capacity; treatment failure; treatment outcome; video assisted thoracoscopic surgery; clinical trial; drug effect; fibrosing alveolitis; lung function test; middle aged; pathophysiology; severity of illness index; time factor; vital capacity, Aged; Anti-Inflammatory Agents, Non-Steroidal; Female; Humans; Idiopathic Pulmonary Fibrosis; Male; Middle Aged; Pyridones; Respiratory Function Tests; Retrospective Studies; Severity of Illness Index; Time Factors; Treatment Outcome; Vital Capacity
DOI:
10.1016/j.pupt.2017.08.011
