IPF: FROM PATHOGENESIS TO CURE Palazzo delle Esposizioni Rome, Italy - May 9-10, 2014 Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, and limited to the lungs. It is characterized by progressive worsening of dyspnea and lung function and is associated with a poor prognosis. The pathogenesis of IPF is still unclear and a complex series of pathological events, many of which shared by other forms of pulmonary and systemic idiopathic fibrosing disorders, have been shown to be involved in the development of the typical Usual Interstitial Pneumonia (UIP) pulmonary lesions. Among them, increased local production of reactive oxygen species, abnormal wound healing in response to multiple microscopic injury, epithelial activation and hyperplasia, presence of cellular de-differentiation and epithelial to mesenchymal transition, imbalance in angiogenetic/angiostatic process, unusual proliferation of mesenchymal cells with matrix deposition, disruption of coagulation pathway and increase of capillary permeability. On the research side, a growing number of studies have been aimed at the identification of the susceptibility gene(s) or the environmental exposures that could drive the pathogenic process of IPF. On the other hand, several controlled clinical trials have been carried out and new effective agents are -or will be soonavailable for the treatment of IPF affected patients. In addition, considerable efforts have been made by scientific societies and associations to improve upon diagnostic and treatment standards for IPF patients and a simpler approach to the diagnosis have emerged from these efforts. Most importantly, interest in IPF basic and clinical research alike is continuously growing. The purpose of this meeting is to provide an update on recent advances in the field of the basic and clinical science of IPF. The most recent advancements in the understanding of the biological processes responsible for IPF pathogenesis will be critically reviewed and the most recent criteria for diagnosing and treating the disease, both in research and real life settings, will be discussed. PRELIMINARY PROGRAM Friday May 9, 2014 09:30 Welcome G. Novelli, Rector University of Roma “Tor Vergata” Introduction C. Saltini SESSION 1 IDIOPATHIC FIBROSING DISORDERS-1 Chairs: F. Pallone, F. Pasqua 10:00 IgG4 related Disease - P. Graziano, San Giovanni Rotondo 10:30 Sarcoidosis - W. P. Drake, Nashville (TN, USA) 11:00 Pulmonary alveolar proteinosis - M. Luisetti, Pavia 11.30 Friedreich's ataxia - R. Testi, Roma 12:00 Coffee Break SESSION 2 PATHOGENESIS OF IPF: INSIGHTS AND PROSPECTIVES Chairs: R. Antonelli, F.Sangiuolo 12:30 13:00 Familial Interstitial Pneumonia - P. Rottoli, Siena Mucin genes - D. Schwartz, Aurora (CO, USA) 13:30 Lunch SESSION 3 VASCULAR ABNORMALITIES IN PULMONARY FIBROSIS Chairs: M. Confalonieri, G. Mazzarella 14.30 15:00 15:30 Vascular abnormalities in smokers and COPD - JA. Barberà, Barcelona Alveolar iron in IIPs: hemorrhage or inhalation? - E. Puxeddu, Roma A common signaling pathway drives fibrosis in lung and skin - V. E. Avvedimento, Napoli 16:00 Coffee Break SESSION 4 IDIOPATHIC FIBROSING DISORDERS-2 Chairs: A. Orlandi, C. Mereu 16.30 Lung fibrosing damage - C. Saltini, Roma 17:00 Asbestosis - A. Ghio, Chapel Hill (NC, USA) 17:30 Hereditary hemochromatosis gene related diseases - E. Corradini, Modena Saturday May 10, 2014 SESSION 5 FROM GUIDELINES TO PATIENT MANAGEMENT: INTERACTIVE SESSION Chairs: C. Agostini, G. Farinelli Introduction 09:00 Radiologically confident IPF, real life - M. Zompatori, Bologna Ground Round - MDD presentation: the possible/inconsistent IPF 09:30 Histological patterns - M. Chilosi, Verona 10:00 The clinical picture - V. Poletti, Forlì 10:30 Coffee Break LECTURE Chair: L. Palombi 11:00 Guidelines implementation: National, European, International? - D. Valeyre, Paris (F) SESSION 6 EVIDENCE BASED TREATMENT Chairs: C. Albera, F. De Michele 11:30 12:00 From Ifigenia to Panther - F. J. Martinez, Ann Arbor (MI, USA) Pirfenidone post-marketing studies – C. Vancheri, Catania 12:30 PH in IPF what to do: ERA, PDE5 inhibitors or palliation? - S. A. Harari, Milano 13:00 Nintedamib rationale and clinical prospective - L.Richeldi, Southampton (UK) 13:30 Closing remarks CHAIRMEN C. Saltini, Roma V. Poletti, Forlì L. Richeldi, Southampton UK SCIENTIFIC COMMITTEE C. Saltini, Roma E. Puxeddu, Roma G. Pezzuto, Roma CONFERENCE VENUE Palazzo delle Esposizioni Via Milano, 9 – Roma CONTINUING MEDICAL EDUCAZION Continuing Medical Education (CME) accreditation will be available for only Italian medical specialist. The event has been insert in the Continuing Medical Education Planning AIPO 2014 Provider 442 ORGANIZING SECRETARIAT Via A. Da Recanate, 2 - 20124 – MILANO Tel. +39 02 36590350 - Fax +39 02 67382337 [email protected] www.aiporicerche.it www.aiponet.it
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