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Primär biliär kolangit

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Sakkunnig:
Definition:
Primär biliär kolangit är en kronisk–progressiv autoimmun kolestatisk sjukdom i de intrahepatiska gallgångarna med inflammation, fibros och till slut cirros med leversvikt och levercancer.
Förekomst:
Incidens 2,1 per en miljon per år, prevalens 285 per miljon invånare. Cirka 80–90 % av patienterna är kvinnor, oftast i medelåldern (40–60 år).
Symtom:
Ibland inga symtom alls. Tidiga tecken är orkeslöshet, besvärande klåda och oförklarlig hyperlipidemi.
Kliniska fynd:
Hepatosplenomegali, hyperpigmentering. Xantomatösa lesioner förekommer. Ikterus och andra tecken på avancerad leversjukdom i ett senare förlopp. 
Diagnos:
Kräver två av följande kriterier: förhöjda alkaliska fosfataser (ALP) under mer än sex månader, förhöjda antikroppar och/eller leverbiopsi med bild som vid primär biliär kolangit.
Behandling:
Ursodeoxicholsyra (UDCA) som förstahands behandling. För UDCA non-responderskan behandling med obeticholsyra (OCA) övervägas. Symptomatisk behandling av klåda och osteoporos.

 

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  • Hanns-Ulrich Marschall, professor och universitetssjukhusöverläkare, Medicinkliniken, Sahlgrenska Universitetssjukhuset, Göteborg
  • Anna Nager, med dr och specialist i allmänmedicin, Karolinska institutet