CASO

Anamnesi | Un uomo di 30 anni si rivolge al medico curante perché da ormai troppo tempo avverte un prurito incoercibile. In anamnesi segnala che soffre di rettocolite ulcerosa che tratta ormai da anni. Il medico lo visita e annota che il paziente presenta clinicamente ittero, perdita di peso, dolorabilità addominale, alvo irregolare, astenia, affaticabilità e altri segni di malassorbimento. In particolare gli prescrive indagini sierologiche. Il paziente si presenta dal medico con i risultati delle analisi e il medico annota iper GammaGT, aumento fosfatasi alcalina, ipercolesterolemia, iperbilirubinemia coniugata. Non convinto chiede una colangiografia. Dopo numerose indagini di conferma non ha più dubbi e convoca il paziente per la poco felice diagnosi. Quale sarà ?


SOLUZIONE

Diagnosi | Colangite sclerosante primaria

La colangite sclerosante primaria è una patologia sclerotica delle vie biliari, probabilmente autoimmune, che clinicamente si associa spesso a malattie infiammatorie intestinali, specie la colite ulcerosa. Non è nota la ragione di questa associazione ma è probabile che il meccanismo patogenetico alla base della colangite sclerosante primaria veda coinvolta una reazione immuno mediata sia umorale che cellulare, forse a partenza delle cellule già coinvolte nell’autoimmunità della colite ulcerosa, le quali riconoscono specifici epitopi sui colangiociti delle vie biliari innestando un classico meccanismo di infiammazione sclerosante.  L’evento finale è che le vie biliari epatiche ed extraepatiche colpite vanno incontro a stenosi e fibrosi periportale dei dotti biliari. Quest’ultima è molto caratteristica all’istologico ed è chiamata ‘a buccia di cipolla’ per l’aspetto concentrico lamellare che assumono gli istiociti epitelioidi responsabili della reazione granulomatosa. La colangite sclerosante quindi è una malattia tipicamente colestatica. Tutti i sintomi sono infatti sintomi tipici della colestasi (es. il prurito, l’ittero colestatico etc.) così come i marker di laboratorio alterati (GGT, fosfatasi alcalina, colesterolo, bilirubina coniugata etc.). La colangite sclerosante inoltre si associa a maggior rischio di neoplasie, soprattutto i colangiocarcinomi e di pancreatiti croniche e carcinoma epatocellulare. Inoltre l’80% dei pazienti ha anticorpi anti-MPO ANCA che sono tipici ma non esclusivi della vasculite dei piccoli vasi detta sindrome di Churg-Strauss. Non si conosce ancora l’entità della patologia. Purtroppo ad oggi non c’è cura per la colangite sclerosante primaria ma una possibilità d’allievo dei sintomi e miglioria della colestasi è dato dalla somministrazione di acido ursodesossicolico responsabile della solubilizzazione del colesterolo e che abbassa la formazione di calcoli biliari (colelitiasi) molto comune nelle colestasi. Inoltre tale terapia abbassa anche il rischio di complicanze a lungo termine legate ad epatopatia cronica come ad es. l’ascite, l’ipertensione portale o la formazione di varici esofagee

Redazione | Nato a Torre del greco il 27/03/1992 5°anno facoltà di medicina e chirurgia Federico II Napoli Appassionato di molte discipline diverse la medicina rappresenta sicuramente la più importante. Non disdegno alcun settore ma le branche che più mi interessano sono quelle della diagnostica clinica, della dermatologia, della ricerca oncologica, della farmacologia, della pediatria e della medicina interna. La mia idea di medico è quella di un lavoro di gruppo che possa essere parte di un team multidisciplinare ben strutturato che garantisca ai pazienti un iter di cura completo ed efficace. Spero un giorno di poter fare esperienze anche in zone di guerra specialmente in medio oriente magari con MSF o Emergency

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