title: A combined therapeutic approach in progressive idiopathic pulmonary fibrosis-pirfenidone as bridge therapy for ex vivo lung transplantation: a case report.
abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial pneumonia of unknown etiology, which is associated with the histopathologic pattern of usual interstitial pneumonia (UIP) and leads to a progressive decrease of respiratory function. The present article describes a case of a 62-year-old ex-smoker referred to our hospital because of IPF. After 2 years of follow-up, the subject experienced a significant worsening of pulmonary function and was enrolled in a lung transplantation program. Afterward, a pharmacological treatment with pirfenidone was started, achieving a stabilization of respiratory function. The patient underwent a single lung transplantation by means of a normothermic ex vivo lung perfusion (EVLP) approach according to the Toronto model. At 20-month evaluation the subject's respiratory function was significantly improved, and quality of life was considerably ameliorated. We believe that an integrated multidisciplinary approach should be considered a key option for the treatment of individuals with IPF.
authors.affiliation: Department of Cardiovascular, Respiratory, Nephrologic, Anesthesiologic and Geriatric Sciences, Sapienza University of Rome, Rome, Italy; Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Rome, Italy. Electronic address: rpaone1023@yahoo.com.
authors.forename: G
authors.initials: G
authors.lastname: Paone
authors.affiliation: Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Rome, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Sebastiani
authors.affiliation: Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Latina, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Ialleni
authors.affiliation: Department of Thoracic Surgery, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Diso
authors.affiliation: Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Latina, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Rose
authors.affiliation: Radiology Unit, S. Camillo-Forlanini Hospital, Rome, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Quagliarini
authors.affiliation: Radiology Unit, S. Camillo-Forlanini Hospital, Rome, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Ialongo
authors.affiliation: Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Latina, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Peruzzi
authors.affiliation: Department of Thoracic Surgery, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Venuta
authors.affiliation: Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Latina, Italy; Department of AngioCardioNeurology, Neuromed, Pozzilli, Isernia, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Frati
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D018450:Disease Progression; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000628:therapy; D011728:Pyridones / Q000627:therapeutic use*; D011788:Quality of Life
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone