title: Efficacy of pirfenidone for idiopathic pulmonary fibrosis: An Italian real life study.
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.
authors.affiliation: 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.
authors.forename: S
authors.initials: S
authors.lastname: Harari
authors.affiliation: Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria-Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe MultiMedica, IRCCS, Milan, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Caminati
authors.affiliation: Department of Clinical and Biological Sciences, Center for Rare Pulmonary Disease, San Luigi Gonzaga Medical School, Turin, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Albera
authors.affiliation: Regional Centre for Rare Lung Disease, University of Catania, Catania, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Vancheri
authors.affiliation: Pulmonary Unit, GB Morgagni Hospital, Forlì, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Poletti
authors.affiliation: Clinica Pneumologica, Department of Health Science, University of Milan Bicocca, AO San Gerardo, Monza, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Pesci
authors.affiliation: Unit of Respiratory Disease, University Hospital, Modena, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Luppi
authors.affiliation: Respiratory Diseases Unit, Department of Medicine, "Tor Vergata" University Hospital, Roma, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Saltini
authors.affiliation: Department of Clinical and Experimental Medicine, University of Padua, Padua, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Agostini
authors.affiliation: Respiratory Diseases and Lung Transplant Unit, Department of Internal and Specialistic Medicine, AOUS, Siena, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Bargagli
authors.affiliation: Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Roma, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Sebastiani
authors.affiliation: Division of Pneumology, Department of Respiratory Diseases, High Speciality Hospital "V. Monaldi" Naples and University of Naples Federico II, Naples, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Sanduzzi
authors.affiliation: Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria-Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe MultiMedica, IRCCS, Milan, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Giunta
authors.affiliation: Department of Pneumology, University Hospital of Trieste, Trieste, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Della Porta
authors.affiliation: Department of Clinical and Biological Sciences, Center for Rare Pulmonary Disease, San Luigi Gonzaga Medical School, Turin, Italy.
authors.forename: G P
authors.initials: GP
authors.lastname: Bandelli
authors.affiliation: Regional Centre for Rare Lung Disease, University of Catania, Catania, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Puglisi
authors.affiliation: Pulmonary Unit, GB Morgagni Hospital, Forlì, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Tomassetti
authors.affiliation: Clinica Pneumologica, Department of Health Science, University of Milan Bicocca, AO San Gerardo, Monza, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Biffi
authors.affiliation: Unit of Respiratory Disease, University Hospital, Modena, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Cerri
authors.affiliation: Respiratory Diseases Unit, Department of Medicine, "Tor Vergata" University Hospital, Roma, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Mari
authors.affiliation: Department of Clinical and Experimental Medicine, University of Padua, Padua, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Cinetto
authors.affiliation: Respiratory Diseases and Lung Transplant Unit, Department of Internal and Specialistic Medicine, AOUS, Siena, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Tirelli
authors.affiliation: Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Roma, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Farinelli
authors.affiliation: Division of Pneumology, Department of Respiratory Diseases, High Speciality Hospital "V. Monaldi" Naples and University of Naples Federico II, Naples, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Bocchino
authors.affiliation: Department of Molecular and Translational Medicine, University of Brescia, Italy; IRCCS MultiMedica, Milano, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Specchia
authors.affiliation: Department of Pneumology, University Hospital of Trieste, Trieste, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Confalonieri
mesh_terms: D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008297:Male; D011728:Pyridones / Q000008:administration & dosage*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone
keywords: IPF; Pirfenidone; Therapy