Current treatments of primary sclerosing cholangitis.
Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic disease characterized by hepatic inflammation and obliterative fibrosis, resulting in both intra- and extra-hepatic bile duct strictures. End-stage liver disease and bile duct carcinoma represent frequent complications. Incidence and prevalence of PSC in USA have been recently estimated as 0.9 per 100,000 person-years, and 1-6 per 100,000 person-years, respectively. Major diagnostic criteria include the presence of multifocal strictures, beadings of bile ducts, and compatible biochemical profile, once excluded secondary causes of cholangitis. Since the aetiology of PSC remains poorly defined, medical therapy is currently limited to symptom improvement and prolonged survival. Ursodeoxycholic acid (UDCA), corticosteroids and immunosuppressants have been proposed alone or in combination to improve the clinical outcome. In selected cases, surgical or endoscopic procedures need to be considered. Orthotopic liver transplantation (OLT) is at the moment the only definitive approach although disease relapse has been reported. In this article the state of the art in PSC treatment and future promises in this field are reviewed.
Vacca
M
M
Clinica Medica "A. Murri", Department of Internal Medicine and Public Medicine (DIMIMP), University of Bari, Italy.
Krawczyk
M
M
Petruzzelli
M
M
Sasso
R C
RC
van Erpecum
K J
KJ
Palasciano
G
G
van Berge-Henegouwen
G P
GP
Moschetta
A
A
Portincasa
P
P
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery*; D006801:Humans; D000960:Hypolipidemic Agents / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016031:Liver Transplantation; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D002756:Cholagogues and Choleretics; D000960:Hypolipidemic Agents; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid