S Harari1, A Caminati2, C Albera3, C Vancheri4, V Poletti5, A Pesci6, F Luppi7, C Saltini8, C Agostini9, E Bargagli10, A Sebastiani11, A Sanduzzi12, V Giunta2, R Della Porta13, G P Bandelli3, S Puglisi4, S Tomassetti5, A Biffi6, S Cerri7, A Mari8, F Cinetto9, F Tirelli10, G Farinelli11, M Bocchino12, C Specchia14, M Confalonieri13. 1. Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria-Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe MultiMedica, IRCCS, Milan, Italy. Electronic address: sharari@hotmail.it. 2. Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria-Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe MultiMedica, IRCCS, Milan, Italy. 3. Department of Clinical and Biological Sciences, Center for Rare Pulmonary Disease, San Luigi Gonzaga Medical School, Turin, Italy. 4. Regional Centre for Rare Lung Disease, University of Catania, Catania, Italy. 5. Pulmonary Unit, GB Morgagni Hospital, Forlì, Italy. 6. Clinica Pneumologica, Department of Health Science, University of Milan Bicocca, AO San Gerardo, Monza, Italy. 7. Unit of Respiratory Disease, University Hospital, Modena, Italy. 8. Respiratory Diseases Unit, Department of Medicine, "Tor Vergata" University Hospital, Roma, Italy. 9. Department of Clinical and Experimental Medicine, University of Padua, Padua, Italy. 10. Respiratory Diseases and Lung Transplant Unit, Department of Internal and Specialistic Medicine, AOUS, Siena, Italy. 11. Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Roma, Italy. 12. Division of Pneumology, Department of Respiratory Diseases, High Speciality Hospital "V. Monaldi" Naples and University of Naples Federico II, Naples, Italy. 13. Department of Pneumology, University Hospital of Trieste, Trieste, Italy. 14. Department of Molecular and Translational Medicine, University of Brescia, Italy; IRCCS MultiMedica, Milano, Italy.
Abstract
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.
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.