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LA COLESTASI Diagnosi E Terapia Basata Sull’Evidenza Linee Guida LA COLESTASI Diagnosi e terapia basata sull’evidenza Linee guida a cura della Commissione “Colestasi” dell’Associazione Italiana per lo Studio del Fegato Redatto da: Mario Angelico (coordinatore), Domenico Alvaro, Cristiana Barbera, Antonio Benedetti, Maria Antonia Bianco, Livio Cipolletta, Michele Colledan, Carla Colombo, Guido Costamagna, Davide Festi, Annarosa Floreani, Giovanni Galatola, Bruno Gridelli, Pietro Invernizzi, Paola Loria, Giuseppe Mazzella, Layos Okolicsanyi, Antonio Orlacchio, Floriano Rosina, Aurelio Sonzogni, Mario Strazzabosco. DOCUMENTI ELABORATI DALLE COMMISSIONI SCIENTIFICHE (RACCOLTA 1996-2001) Abbreviazioni usate nel testo: AMA: anticorpi anti-mitocondrio ANA: anticorpi anti-nucleo ASMA: anticorpi anti-muscolo liscio ANCA: anticorpi anti-citoplasma dei neutrofili AVB: atresia delle vie biliari OLT: trapianto di fegato (orthotopic liver transplantation) MRS: Mayo Risk Score (per CBP) CBP: cirrosi biliare primitiva CSP: colangite sclerosante primitiva CPRE: colangio-pancreatografia retrograda per-endoscopica CPRM: colangio-pancreatografia a risonanza magnetica CPT: colangiografia transepatica percutanea EUS: endosonografia US: ultrasonografia (ecografia) VBP: via biliare principale PPV: valore predittivo positivo NPV: valore predittivo negativo UDCA: acido ursodesossicolico TC: tomografia computerizzata SE: sfinterotomia endoscopica DCVB: dilatazioni congenite delle vie biliari CSA: colangite sclerosante autoimmune DNB: drenaggi nasobiliari PTDB drenaggi biliari dopo trapianto al fegato RCT: randomized controlled trial 288 DOCUMENTI ELABORATI DALLE COMMISSIONI SCIENTIFICHE (RACCOLTA 1996-2001) INDICE Capitolo 1: 1. INTRODUZIONE 291 - Obiettivi ed aspetti metodologici - Definizione di colestasi - Classificazione delle colestasi - Algoritmi diagnostici Capitolo 2: 2. LE COLESTASI INTRAEPATICHE DELL'ADULTO 296 - Sindromi da vanificazione dei dotti biliari non genetiche • Cirrosi biliare primitiva (CBP) • Colangite autoimmune (o CBP AMA-negativa) • Duttopenia idiopatica dell'adulto • Colangite sclerosante primitiva (CSP) • Sindromi da "overlap" - Colestasi infettive - Colestasi da farmaci - Colestasi gravidica - Colestasi neoplastiche - Colestasi post-trapianto Capitolo 3: 3. LE COLESTASI INTRAEPATICHE PEDIATRICHE 336 - Atresia delle vie biliari - Dilatazioni congenite - Colangite sclerosante primitiva - Colestasi su base genetica • Deficit di alfa1-antitripsina • Sindrome di Alagille • Colestasi intraepatiche familiari progressive (PFIC) • Errori nella sintesi degli acidi biliari • Colestasi intraepatica benigna ricorrente (BRIC) • Fibrosi cistica • Patologia della placca duttale • Epatite neonatale idiopatica - Terapia medica della colestasi cronica del bambino - Terapia chirurgica della colestasi cronica del bambino Capitolo 4: 4. LE COLESTASI EXTRAEPATICHE (OSTRUTTIVE) 362 - Inquadramento clinico - Coledocolitiasi - Stenosi benigne - Stenosi maligne 289 DOCUMENTI ELABORATI DALLE COMMISSIONI SCIENTIFICHE (RACCOLTA 1996-2001) Capitolo 5: 5. USO RAZIONALE DEGLI STRUMENTI 374 DIAGNOSTICI E TERAPEUTICI - Tests bioumorali, sierologici e genetici - Indagini strumentali non invasive • Ecografia (US) • Colangio-Pancreatografia a Risonanza Magnetica (CPRM) • Scintigrafia con HIDA - Procedure strumentali invasive diagnostiche e terapeutiche • Ecoendoscopia (EUS) • Colangio-Pancreatografia-Retrograda-perEndoscopica (CPRE) • Sfinterotomia endoscopica • Inserzione di endoprotesi • Drenaggi naso-biliari e percutanei • Profilassi antibiotica nelle procedure interventistiche • Biopsia epatica Capitolo 6: 6. IL TRATTAMENTO DEI SINTOMI 393 - Prurito - Malassorbimento - Osteopenia - Dolore biliare di tipo colico Capitolo 7: 7. VARIE 399 - Indicazioni alla colecistectomia laparoscopica - Indicazioni al trapianto di fegato 290 DOCUMENTI ELABORATI DALLE COMMISSIONI SCIENTIFICHE (RACCOLTA 1996-2001) INTRODUZIONE 1. OBIETTIVI ED ASPETTI METODOLOGICI La Commissione "Colestasi" dell'Associazione Italiana Studio Fegato (AISF) si è insediata nel settembre 1999. Sono stati invitati a farne parte i soci AISF attivi nel settore della ricerca clinica e di base in tema di cole- stasi ed altri esperti esterni individuati dalla Commissione. E' stato così costituito un panel di esperti mul- tidisciplinare, comprendente specialisti in gastroenterologia, epatologia, pediatria, endoscopia digestiva, chirurgia epato-biliare, radiologia ed anatomia patologica. L'obiettivo che la Commissione si è proposto è stato quello di elaborare un documento tecnico, basato sui principi della medicina basata sull'evidenza (evidence-based-medicine), da utilizzarsi come strumento di riferimento nella gestione clinica delle diverse forme di colestasi. Sono stati individuati tre obiettivi specifici principali: 1) definire i criteri di diagnosi e terapia delle diverse forme di colestasi intra-epatica ed extraepatica dell'età adulta e dell'età pediatrica; 2) definire l'uso razionale delle diverse metodiche diagnostiche e terapeutiche; 3) definire le modalità di trattamento dei sintomi. Nell'elaborazione del documento tecnico la Commissione ha proceduto ad effettuare preliminarmente una revisione sistematica della letteratura tramite MEDLINE, che è stata affidata ad uno o più esperti per cia- scun argomento. Quindi sono stati elaborati drafts preliminari su ciascun tema che sono stati inviati di volta in volta per e-mail a tutti i componenti della Commissione per essere sottoposti a revisione. Infine è stato elaborato il documento finale, che è stato approvato da tutta la Commissione in più sedute collegiali. In secondo luogo la Commissione ha utilizzato l'elaborato tecnico per stendere, quando possibile, linee guida (o raccomandazioni) per la diagnosi e terapia di alcune forme di colestasi, rivolte sia al medico di base che allo specialista. Il documento tecnico e le raccomandazioni hanno tenuto conto, ove possibile, dei livelli di certezza definiti da Ball et al. (1), illustrati nella tabella 1.1. L'applicazione di tali livelli è stata generalmente semplice e completa per la valutazione di studi terapeutici, mentre è stata più difficile per la valutazione di metodiche diagnostiche. Le linee guida elaborate non intendono essere rigidi protocolli ma solo uno strumento con il quale il medico possa confrontarsi in relazione alle sue scelte diagnostiche e terapeutiche. Sia nel documento tecnico che nelle linee guida propriamente dette è indicato, quando possibile, il livello di evidenza scientifi- ca corrispondente ad ogni specifica raccomandazione, unitamente ai riferimenti bibliografici pertinenti. La Commissione ha preso atto nel corso dei lavori che per alcuni argomenti trattati non vi sono meta-ana- lisi, né studi randomizzati o controllati e quindi il livello di evidenza scientifica è modesto. Ciò vale soprat- tutto per le metodiche diagnostiche più innovative e per le patologie meno frequenti, come ad esempio le colestasi pediatriche. Sorprendentemente, però, mancano evidenze scientifiche robuste anche per alcune patologie comuni o per metodiche diagnostiche e terapeutiche di uso routinario. Ne consegue che le affer- mazioni contenute nel presente documento si basano spesso sull'opinione di esperti o derivano da studi non controllati o di modesta qualità. Per limitare la possibilità di errori ed il rischio di autoreferenzialità si è fatto ampio ricorso nell'elaborazione delle linee guida al principio della multidisciplinarietà. Quando possibile, anche la valutazione dei tests diagnostici è stata effettuata tenendo conto di criteri internazio- nalmente riconosciuti (2,3). Infine, la Commissione ha cercato di mettere in evidenza le aree di dissenso o controversia ed i settori per i quali il rapido evolversi della tecnologia biomedica fa prevedere la necessità di rivalutare le attuali raccomandazioni in un prossimo futuro. 291 DOCUMENTI ELABORATI DALLE COMMISSIONI SCIENTIFICHE (RACCOLTA 1996-2001) BIBLIOGRAFIA 1. Ball C, Sackett D, Phillips B, Haynes B, Strass S. Levels of evidence and grade of recommendation (http://www.cebm.jr2.ox.ac.uk/docs/levels.html) 2. Schoenfeld et al. Evidence Based Gastroenterology. Gastroenterology 1998;114:1318-1325) 3. Schoenfeld et al. Evidence-Based Gastroenterology. Gastroenterology 1999;116:1230-1237) Tabella 1.1 LIVELLI DI EVIDENZA (ref. n. 1) Grado di certezza Metodologia di valutazione A 1a Metanalisi con omogeneità tra RCTs A 1b RCT singolo con stretti limiti di confidenza A 1c Metanalisi di studi di coorte con omogeneità B 2b Studio individuale di coorte, o RCT di non alta qualità (f.u. <80% pts) B 3a Metanalisi con eterogeneità B 3b Studio individuale di tipo caso-controllo C 4 Studi osservazionali, caso-controllo di non alta qualità D 5 Opinione di esperti senza riferimento ad esplicito metodo di valutazione 2. DEFINIZIONE DI COLESTASI La colestasi è una sindrome clinica di espressione e gravità variabile conseguenza di una alterazione della secrezione o del normale deflusso di bile nel duodeno. La definizione di colestasi varia a seconda del punto di vista primario che si considera. Dal punto di vista biochimico il comune denominatore è l'elevazione sie- rica di enzimi specifici di colestasi, come la fosfatasi alcalina e la gamma-glutamiltransferasi, e la riten- zione nel sangue di composti colefilici, cioè normalmente secreti nella bile, come i sali biliari e la bilirubi- na. Dal punto di vista istopatologico, la colestasi è definitita dalla presenza di segni di colatostasi e/o bili- rubinostasi. Dal punto di vista clinico la colestasi è caratterizzata dalla presenza di uno o più sintomi di intensità variabile che
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