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Antimitochondrial antibodies
100%
3/3
Antiendomysial antibodies
0%
0/3
Anti-Jo-1 antibodies
Antihistone antibodies
Anticentromere antibodies
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This patient's presentation is consistent with primary biliary cirrhosis. Elevated antimitochondrial antibodies may also be found in this disease. Primary biliary cirrhosis is a rare autoimmune disease often found in a middle aged woman. Inflammatory infitrates cause destruction of the bile ducts in the portal triads, hampering the excretion of bile salts. This patient's symptoms of pruritis and jaundice are characteristic. Pruritis is caused by bile salt deposition in the skin. Antimitochondrial antibodies are positive in most patients, but the test only has a sensitivity of 90% so a negative result must be confirmed with biopsy. Definitive treatment is liver transplant. However, supportive therapy may be administered using ursodeoxycholic acid which can decrease itching. Selmi et al. discuss diagnosis, treatment, and management of primary biliary cirrhosis. Concordance rates in monozygotic twins, familial prevalence, and genetic associations underscore the importance of genetic factors in the etiology of the disease, 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. This is possibly attributable to augmented testing of liver biochemistry rather than a rise in disease incidence. Hirschfield et al. discuss the immunobiology and pathophysiology of primary biliary cirrhosis. The disease is marked by an overwhelming female predominance and production of a multilineage immune response to mitochondrial autoantigens causing inflammation of small bile ducts, and in some patients the development of fibrosis and cirrhosis. Illustration A depicts a biopsy specimen of primary biliary cirrhosis, notable for a significant lymphocytic infiltrate. Incorrect Answers: Answer 2: Antiendomysial antibodies are found in Celiac disease. Answer 3: Anti-Jo-1 antibodies are found in polymyositis and dermatomyositis. Answer 4: Antihistone antibodies are found in drug-induced lupus. Answer 5: Anticentromere antibodies are found in scleroderma (CREST).
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