Primary biliary cirrhosis

被引:326
作者
Selmi, Carlo [2 ]
Bowlus, Christopher L. [3 ]
Gershwin, M. Eric [4 ]
Coppel, Ross L. [1 ]
机构
[1] Monash Univ, Dept Microbiol, Clayton, Vic 3800, Australia
[2] Univ Milan, Dept Translat Med, IRCCS Ist Clin Humanitas, Milan, Italy
[3] Univ Calif Davis, Div Gastroenterol & Hepatol, Davis, CA 95616 USA
[4] Univ Calif Davis, Div Rheumatol Allergy & Clin Immunol, Davis, CA 95616 USA
关键词
URSODEOXYCHOLIC-ACID THERAPY; RANDOMIZED CONTROLLED-TRIALS; LONG-TERM SURVIVAL; REGULATORY T-CELLS; ANTIMITOCHONDRIAL ANTIBODIES; CLINICAL-FEATURES; ANTINUCLEAR ANTIBODIES; BIOCHEMICAL RESPONSE; NATURAL-HISTORY; LIVER-DISEASE;
D O I
10.1016/S0140-6736(10)61965-4
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Primary biliary cirrhosis is a chronic liver disease characterised by intrahepatic bile-duct destruction, cholestasis, and, in some cases, cirrhosis. Evidence supporting the autoimmune nature of this disorder includes the appearance of highly specific antimitochondrial antibodies (AMAs) and autoreactive T cells. Concordance rates in monozygotic twins, familial prevalence, and genetic associations underscore the importance of genetic factors, whereas findings of epidemiological studies and murine models suggest a possible role for exogenous chemicals and infectious agents through molecular mimicry. The incidence of primary biliary cirrhosis has increased over recent decades, possibly attributable to augmented testing of liver biochemistry rather than a rise in disease incidence. AMAs remain the hallmark of diagnosis in most cases and allow detection of asymptomatic patients. Symptomatic individuals usually present with either pruritus or fatigue and, more rarely, with either jaundice or complications of cirrhosis. The prognosis of primary biliary cirrhosis has improved because of early diagnosis and use of ursodeoxycholic acid, the only established medical treatment for this disorder. Although not a cure, treatment can slow disease progression and delay the need for liver transplantation. However, some patients do not respond adequately to ursodeoxycholic acid and might need alternative therapeutic approaches.
引用
收藏
页码:1600 / 1609
页数:10
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