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QUADERNI Dell’Italian Journal NE 2017 – VOLU M of Medicine volume 5 ISSUE 7 2017 settembre SSUE 7 I E 5 – M QUADERNI dell’Italian Journal NE 2017 – VOLU ICI of Medicine A Journal of Hospital and Internal Medicine AN JOURNAL OF MED I Editor in Chief The official journal of the Federation of Associations Roberto Nardi of Hospital Doctors on Internal Medicine (FADOI) – ITAL I Non-commercial use only UADERN Q Le malattie infiammatorie immuno-mediate (IMID) di interesse internistico: fisiopatologia, aspetti clinici e prospettive di terapia Guest Editors: A. Fontanella, R. Nardi, M. Masina, G. Uomo PAGEPress - Scientific Publications Via A. Cavagna Sangiuliani, 5 - 27100 Pavia - Italy Phone +39 0382 464340 - Fax +39 0382 34872 www.pagepress.org Q www.italjmed.org SEGRETERIA NAZIONALE Via Felice Grossi Gondi, 49 - 00162 Roma Tel. +39.06.85355188 - Fax: +39.06.85345986 E-mail: [email protected] Società Scientifica FADOI - Organigramma PRESIDENTE NAZIONALE Andrea Fontanella, Napoli, Italy PRESIDENTE ELETTO COORDINATORE COMMISSIONE GIOVANI Dario Manfellotto, Roma, Italy Flavio Tangianu, Oristano, Italy PAST PRESIDENT MEMBRO FISM E RESPONSABILE PER L’INNOVAZIONE IN MEDICINA INTERNA Mauro Campanini, Novara, Italy Antonino Mazzone, Legnano (MI), Italy SEGRETARIO ITALIAN JOURNAL OF MEDICINE Micaela La Regina, La Spezia, Italy Editor in Chief Giorgio Vescovo, Padova, Italy SEGRETARIO VICARIO RESPONSABILE DEI QUADERNI DELL’IJM Andrea Montagnani, Grosseto, Italy E EDIZIONI ON LINE Roberto Nardi, Bologna, Italy STAFF DI SEGRETERIA Comunicazione Paola Gnerre, Savona, Italy DELEGATO FADOI ITALIAN STROKE ORGANIZATION Ricerca Roberta Re, Novara, Italy E CONSULTA CARDIOVASCOLARE Formazione Maurizia Gambacorta, Todi (PG), Italy Michele Stornello, Siracusa, Italy TESORIERE RAPPORTI CON EFIM David Terracina, Roma, Italy Gualberto Gussoni, Milano, Italy Ombretta Para, Firenze, Italy STAFF DI TESORERIA Giorgio Vescovo, Padova, Italy Francesco D’Amore, Roma, Italy RESPONSABILE SITO NAZIONALE E COMUNICAZIONE PRESIDENTE FONDAZIONE FADOI Salvatore Lenti, Arezzo, Italy Mauro Campanini, Novara, Italy RESPONSABILE SISTEMA GESTIONE QUALITÀ COORDINATORE Franco Berti, Roma, Italy Giuseppe Augello, Canicattì (AG), Italy RESPONSABILE CLINICAL COMPETENCE DIPARTIMENTO PER LA RICERCA CLINICA “CENTRO STUDI FADOI” Antonino Mazzone, Legnano (MI), Italy Non-commercial use only Direttore Giancarlo Agnelli, Perugia, Italy RESPONSABILE AREA ECOGRAFIA Direttore vicario Francesco Dentali, Varese, Italy Francesco Cipollini, Ascoli Piceno, Italy Marcello Romano, La Spezia, Italy DIPARTIMENTO PER LA FORMAZIONE E AGGIORNAMENTO RESPONSABILE MEDICINA DI GENERE Direttore Mauro Silingardi, Guastalla (RE), Italy Cecilia Politi, Isernia, Italy COORDINAMENTO FORMAZIONE RESPONSABILE AREA NUTRIZIONE AREA CENTRO-NORD Roberto Risicato, Siracusa, Italy Francesco Orlandini, La Spezia, Italy Luciano Tramontano, Praia a Mare (CS), Italy COORDINAMENTO FORMAZIONE RESPONSABILE AREA DI CLINICAL GOVERNANCE AREA CENTRO SUD Giovanni Iosa, Cesenatico (FC), Italy Generoso Uomo, Napoli, Italy Stefano De Carli, Udine, Italy Italian Journal of Medicine A Journal of Hospital and Internal Medicine Fondazione FADOI - Organigramma PRESIDENTE FONDAZIONE Mauro Campanini, Novara, Italy COORDINATORE STAFF AREA ORGANIZZATIVA IN MEDICINA INTERNA Giuseppe Augello, Canicattì (AG), Italy Marco Candela, Jesi (AN), Italy Giovanni Mathieu, Pinerolo (TO), Italy DIPARTIMENTO PER LA RICERCA CLINICA Valentino Moretti, San Daniele del Friuli (UD), Italy CENTRO STUDI FADOI Filomena Pietrantonio, Brescia, Italy Elisa Romano, La Spezia, Italy Direttore Giancarlo Agnelli, Perugia, Italy Direttore vicario Francesco Dentali, Varese, Italy DIRETTORE DIPARTIMENTO PER LA FORMAZIONE E AGGIORNAMENTO SEGRETERIA Grazia Panigada, Pescia (PT), Italy Mauro Silingardi, Guastalla (RE), Italy RESPONSABILE AREA MALATTIE CARDIOVASCOLARI COORDINAMENTO DI AREA FORMATIVA CENTRO-NORD Paolo Verdecchia, Assisi (PG), Italy Francesco Orlandini, La Spezia, Italy STAFF AREA MALATTIE CARDIOVASCOLARI COORDINAMENTO DI AREA FORMATIVA CENTRO SUD Cecilia Becattini, Perugia, Italy Generoso Uomo, Napoli, Italy Pierpaolo Di Micco, Napoli, Italy Fernando Gallucci, Napoli, Italy STAFF DIPARTIMENTO PER LA FORMAZIONE Alessandro Squizzato, Varese, Italy E AGGIORNAMENTO Roberto Frediani, Domodossola (VB), Italy RESPONSABILE AREA MALATTIE INFETTIVE Marco Grandi, Sassuolo (MO), Italy Ercole Concia, Verona, Italy SEGRETERIA DIPARTIMENTO PER LA FORMAZIONE E STAFF AREA MALATTIE INFETTIVE AGGIORNAMENTO Anna Maria Azzini, Verona, Italy Luigi Magnani, Voghera (PV), Italy Gianluca Giuri, Castelnovo ne’ Monti (RE), Italy Matteo Giorgi Pierfranceschi, Piacenza, Italy BOARD SCIENTIFICO Carlo Tascini, Pisa, Italy Clelia Canale, Reggio Calabria, Italy RESPONSABILE AREA MALATTIE RESPIRATORIE Fabrizio Colombo, Milano, Italy Giuseppe De Matthaeis, Città Sant’Angelo (PE), Italy Leonardo Fabbri, Reggio Emilia, Italy Massimo Giusti, Torino, Italy Luca Masotti, Cecina (LI), Italy STAFF AREA MALATTIE RESPIRATORIE Nicola Mumoli, Livorno, Italy Maurizio Ongari, Porretta Terme (BO), Italy Bianca Beghè, Reggio Emilia, Italy Ruggero Pastorelli, Colleferro (RM), Italy Piera Boschetto, Ferrara, Italy Fulvio Pomero, Savigliano (CN), Italy Gaetano Cabibbo, Modica (RG), Italy Roberto Risicato, Siracusa, Italy Francesco Corradi, Firenze, Italy Antonio Sacchetta, Conegliano (TV), Italy Francesco Ventrella, Cerignola (FG), Italy Giancarlo Tintori, Pisa, Italy Non-commercial use only RESPONSABILE AREA MALATTIE REUMATOLOGICHE COMMISSIONE TECNICHE E METODICHE INNOVATIVE Carlo Salvarani, Reggio Emilia, Italy DI FORMAZIONE E VERIFICA STAFF AREA MALATTIE REUMATOLOGICHE Responsabile dell’Innovazione in Medicina Interna Antonino Mazzone, Legnano (MI), Italy Laura Morbidoni, Senigallia (AN), Italy Nicolò Pipitone, Reggio Emilia, Italy STAFF Tito D’Errico, Napoli, Italy Francesco Dentali, Varese, Italy RESPONSABILE AREA MALATTIE METABOLICHE Andrea Montagnani, Grosseto, Italy Filippo Pieralli, Firenze, Italy Roberto Vettor, Padova, Italy DIRETTORE SCIENTIFICO FONDAZIONE FADOI STAFF AREA MALATTIE METABOLICHE Gualberto Gussoni, Grosseto, Italy Tiziana Attardo, Agrigento, Italy Giovanni Gulli, Savigliano (CN), Italy REFERENTI SLOW MEDICINE Ada Maffettone, Napoli, Italy Maurizio Nizzoli, Forlì, Italy Roberto Frediani, Domodossola (VB), Italy Luigi Lusiani, Castelfranco Veneto (TV), Italy RESPONSABILE AREA ORGANIZZATIVA IN MEDICINA INTERNA RESPONSABILE AREA DOLORE Antonio Greco, San Giovanni Rotondo (FG), Italy Domenico Panuccio, Bologna, Italy QUADERNI - Italian Journal of Medicine 2017; volume 5(7) QUADERNI - Italian Journal of Medicine LE MALATTIE INFIAMMATORIE IMMUNO-MEDIATE (IMID) DI INTERESSE INTERNISTICO: FISIOPATOLOGIA, ASPETTI CLINICI E PROSPETTIVE DI TERAPIA Guest Editors: A. Fontanella, R. Nardi, M. Masina, G. Uomo RASSEGNE Le IMID: inquadramento introduttivo . 1 A. Fontanella, G. Uomo Infiammazione e IMID . 4 T. d’Errico, M. Laccetti Malattia IgG4-relata . 19 C. Mastrobuoni, G. Uomo IMID in ematologia . 25 F. Rezzonico, A. Mazzone IMID in reumatologia . 30 A. Parisi, R. Buono, R. Russo, G. Uomo IMIDs in neurologia. 36 G.T. Maniscalco, C. Florio IMID in diabetologia . 57 R. Nicosia, C. Ricordi IMIDs in endocrinologia . 67 M.R. Poggiano, V. Nuzzo Malattie infiammatorie intestinali . 75 M. Salice, L. Calandrini, C. Praticò, M. Mazza, A. Calafiore, G. Carini, C. Calabrese, A. Belluzzi, F. Rizzello, P. Gionchetti, M. Campieri La malattia celiaca nel terzo millennio: nuove prospettive su patogenesi, clinica, diagnosi e terapia . 98 Non-commercialG. Caio, F. Giancola, R. De Giorgio, use U. Volta only Malattie epato-biliari autoimmuni. 115 M. Visconti, L. Fontanella, G. Marino Marsilia Pancreatiti autoimmuni. 138 P.G. Rabitti, R. Boni IMID e tumori . 145 F. Gallucci La sindrome infiammatoria da ricostruzione immune. 155 I. Ronga, G. Uomo Le malattie autoinfiammatorie . 161 M. Gattorno, A. Brucato Il rischio cardio-vascolare nelle IMIDs . 172 A. Fontanella, P. Gnerre, R. Nardi QUADERNI - Italian Journal of Medicine 2017; volume 5(7) QUADERNI - Italian Journal of Medicine Quale ruolo degli inibitori delle PCSK9 in Medicina Interna nella prevenzione cardio-vascolare in alternativa alle terapie tradizionali? . 179 P. Gnerre, P. Zuccheri, M. Campanini, G. Pinna, R. Nardi Trombo-embolismo venoso e malattie autoimmuni sistemiche . 188 A. Fontanella, P. Gnerre, R. Nardi IMID nell’anziano: cenni sul trattamento. 194 M. Masina Farmaci biosimilari e farmaci innovativi in reumatologia: quale futuro? . 200 M. Todoerti, C. Montecucco Non-commercial use only QUADERNI - Italian Journal of Medicine 2017; volume 5(7):1-3 Le IMID: inquadramento introduttivo Andrea Fontanella,1 Generoso Uomo2 1UOSC Medicina Interna, Ospedale Fatebenefratelli, Napoli; 2UOSC Medicina Interna 3, AORN Cardarelli, Napoli, Italia nell’animale da esperimento e nell’uomo sostanzial- Introduzione mente attestano che un disordine/interruzione/devia- Le malattie infiammatorie immunomediate (IMID) zione di questi fini processi mediati dalle cellule B può identificano un gruppo di condizioni patologiche appa- portare potenzialmente ad una ampia varietà di malattie rentemente non collegate tra loro ma che condividono autoimmuni. Anche i linfociti T sono naturalmente 1 coinvolti in queste complesse inter-relazioni, in parti- un comune meccanismo infiammatorio. Le IMID po- 3 trebbero in alternativa essere chiamate anche malattie colare le γδ T-cells. Si tratta di un sottotipo di linfociti da disregolazione citochinica in quanto, sebbene per T responsabili del mantenimento della omeostasi e della molte di esse la eziologia
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