Clinical features and management of primary biliary cirrhosis.
Primary biliary cirrhosis (PBC), which is characterised by progressive destruction of intrahepatic bile ducts, is not a rare disease since both prevalence and incidence are increasing during the last years mainly due to the improvement of case finding strategies. The prognosis of the disease has improved due to both the recognition of earlier and indolent cases, and to the wide use of ursodeoxycholic acid (UDCA). New indicators of prognosis are available that will be useful especially for the growing number of patients with less severe disease. Most patients are asymptomatic at presentation. Pruritus may represent the most distressing symptom and, when UDCA is ineffective, cholestyramine represents the mainstay of treatment. Complications of long-standing cholestasis may be clinically relevant only in very advanced stages. Available data on the effects of UDCA on clinically relevant end points clearly indicate that the drug is able to slow but not to halt the progression of the disease while, in advanced stages, the only therapeutic option remains liver transplantation.
Dipartimento di Medicina Interna, Polo Universitario, Ospedale San Paolo, Via di Rudini 8, Milano 20142, Italy. crosihsp@unimi.it
Andrea
A
Crosignani
Pier-Maria
PM
Battezzati
Pietro
P
Invernizzi
Carlo
C
Selmi
Elena
E
Prina
Mauro
M
Podda
D000818:Animals; D001327:Autoimmune Diseases / Q000150:complications* / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D001851:Bone Diseases, Metabolic / Q000209:etiology / Q000628:therapy; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D002792:Cholestyramine Resin / Q000627:therapeutic use; D018450:Disease Progression; D005221:Fatigue / Q000209:etiology / Q000628:therapy; D006801:Humans; D006949:Hyperlipidemias / Q000209:etiology / Q000628:therapy; D006975:Hypertension, Portal / Q000209:etiology / Q000628:therapy; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D016031:Liver Transplantation; D044342:Malnutrition / Q000209:etiology / Q000628:therapy; D011537:Pruritus / Q000209:etiology / Q000628:therapy; D012307:Risk Factors; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D002792:Cholestyramine Resin; D014580:Ursodeoxycholic Acid