Efficacy of pirfenidone for idiopathic pulmonary fibrosis: An Italian real life study. Issue 7 (July 2015)

• Record Type: Journal Article
• Authors: Harari, S.; Caminati, A.; Albera, C.; Vancheri, C.; Poletti, V.; Pesci, A.; Luppi, F.; Saltini, C.; Agostini, C.; Bargagli, E.; Sebastiani, A.; Sanduzzi, A.; Giunta, V.; Della Porta, R.; Bandelli, G.P.; Puglisi, S.; Tomassetti, S.; Biffi, A.; Cerri, S.; Mari, A.; Cinetto, F.; Tirelli, F.; Farinelli, G.; Bocchino, M.; Specchia, C.; Confalonieri, M.
• Is Part Of: Respiratory medicine. Volume 109:Issue 7(2015)
• Page Start: 904
• Page End: 913
• Publication Date: 2015-07
• Subjects: IPF -- Pirfenidone -- Therapy; AE acute exacerbation -- ATS American Thoracic Society -- EMA European Medicine Agency -- ERS European Respiratory Society -- DLCO carbon monoxide diffusing capacity -- FVC forced vital capacity -- IPF idiopathic pulmonary fibrosis -- NAC N-acetylcysteine -- NPP named patient access program -- NSIP non-specific interstitial pneumonia -- PIRF pirfenidone -- UIP usual interstitial pneumonia -- 6MWT 6 minute walk test; Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2015.04.010
• Languages: English
• ISSNs: 0954-6111

Abstract

Summary: Background: In this retrospective Italian study, which involved all major national interstitial lung diseases centers, we evaluated the effect of pirfenidone on disease progression in patients with IPF. Methods: We retrospectively studied 128 patients diagnosed with mild, moderate or severe IPF, and the decline in lung function monitored during the one-year treatment with pirfenidone was compared with the decline measured during the one-year pre-treatment period. Results: At baseline (first pirfenidone prescription), the mean percentage forced vital capacity (FVC) was 75% (35–143%) of predicted, and the mean percentage diffuse lung capacity (DLCO) was 47% (17–120%) of predicted. Forty-eight patients (37.5%) had mild disease (GAP index stage I), 64 patients (50%) had moderate IPF (stage II), and 8 patients (6.3%) had severe disease (stage III). In the whole population, pirfenidone attenuated the decline in FVC (p = 0.065), but did not influence the decline in DLCO (p = 0.355) in comparison to the pre-treatment period. Stratification of patients into mild and severe disease groups based on %FVC level at baseline (>75% and ≤75%) revealed that attenuation of decline in FVC (p = 0.002) was more pronounced in second group of patients. Stratification of patients according to GAP index at baseline (stage I vs. II/III) also revealed that attenuation of decline in lung function was more pronounced in patients with more severe disease. Conclusions: In this national experience, pirfenidone reduced the rate of annual FVC decline (p = 0.065). Since pirfenidone provided significant treatment benefit for patients with moderate-severe disease, our results suggest that the drug may also be effective in patients with more advanced disease.