Antimitochondrial antibody negative primary biliary cirrhosis: a distinct syndrome of autoimmune cholangitis.

Michieletti, Pina; Wanless, Ian R.; Katz, Ariel; Scheuer, Peter J.; Yeaman, Stephen J.; Bassendine, Margaret F.; Palmer, Jeremy M.; Heathcote, E. Jenny

doi:10.1136/gut.35.2.260

Cited by 229 publications

(132 citation statements)

References 30 publications

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“…In this instance, they may be coincidental findings that do not impact on the principal clinical syndrome or management strategy. Alternatively, they may be a weak expression of an overlap syndrome with autoimmune cholangitis, [31][32][33][34] primary biliary cirrhosis, [35][36][37] or primary sclerosing cholangitis. [38][39][40][41][42][43] In this instance, they may identify a mild variant disease that responds well to conventional corticosteroid therapy.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis with incidental histologic features of bile duct injury

2001

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Bile duct changes are atypical of autoimmune hepatitis. Our aims were to assess the frequency and significance of these changes in classical disease. Liver biopsy specimens were reviewed under code from 84 patients who satisfied international scoring criteria for autoimmune hepatitis, and the findings were correlated with clinical features and outcome. Twenty patients (24%) had biliary changes, including 6 with destructive cholangitis, 4 with ductopenia, and 10 with nondestructive cholangitis. Patients with and without bile duct changes had similar laboratory findings. Diagnostic scores for autoimmune hepatitis were lower in patients with bile duct changes (16.6 ؎ 0.6 vs. 19.1 ؎ 0.2, P < .0001). The frequencies of scores sufficient for a definite (80% vs. 97%, P ‫؍‬ .03) or probable diagnosis (20% vs. 3%, P ‫؍‬ .03) were also less in this group. Patients with destructive cholangitis and/or ductopenia responded as well to therapy as patients with nondestructive cholangitis, and outcomes in each group were similar to those of patients without biliary changes. We concluded that biliary changes can occur in classic autoimmune hepatitis, and they are not associated with distinctive clinical features or treatment response.

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Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis with incidental histologic features of bile duct injury

2001

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“…[16][17][18][19][20] These patients have been described as having ''immune cholangitis'' or ''autoimmune cholangitis.'' Most likely they are cases of PBC, except their non-organ-specific antibody profile is more in keeping with that of autoimmune hepatitis; i.e., they have high titer ANA and/or SMA.…”

Section: Ama-negative Pbcmentioning

confidence: 99%

“…In AMA-negative PBC, the IgG fraction is elevated whereas the IgM fraction is less likely to be increased. 18,20 …”

Section: Immunoglobulinsmentioning

confidence: 99%

Management of primary biliary cirrhosis

2000

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Primary biliary cirrhosis (PBC) is a presumed autoimmune disease of the liver, which predominantly affects women once over the age of 20 years. Most cases are diagnosed when asymptomatic (60%). The antimitochondrial antibody is present in serum in most, but not in all, patients with PBC. The disease generally progresses slowly but survival is less than an age-and gender-matched general population. The symptomatic patient may have fatigue, generalized pruritus, portal hypertension, osteoporosis, skin xanthomata, fat soluble vitamin deficiencies, and/or recurrent asymptomatic urinary tract infections. Many nonhepatic autoimmune diseases are found in association with PBC and may prompt initial presentation. To date, immunosuppressive therapy has not been shown to prolong survival in PBC. The hydrophilic bile acid, ursodeoxycholic acid (UDCA), has been shown when given in a dose of 13 to 15 mg/kg daily for up to 4 years to delay the time to liver transplantation or death. This therapy also causes a significant improvement of all the biochemical markers of cholestasis but has no beneficial effects on any of the symptoms or associated disorders. Treatment with UDCA does not obviate the need for liver transplantation. Therapies to prevent complications arising from malabsorption, portal hypertension, and/or osteoporosis are required as well. Good control of pruritus can be achieved in most patients. PBC is diagnosed with increasing frequency, but the agent(s) responsible for this slowly progressive destruction of the interlobular bile ducts remains elusive and hence a specific therapy remains unavailable. (HEPATOLOGY 2000;31:1005-1013.) PREAMBLE

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“…Borderline disease between PBC and AIH, known as overlap syndrome or PBC mixed type is still controversial (4)(5)(6). It is known that the serum levels of transaminase and -globulin seen in overlap syndrome are higher than those seen in pure PBC.…”

Section: Discussionmentioning

confidence: 99%

Rapid-onset Primary Biliary Cirrhosis Resembling Drug-induced Liver Injury

et al. 2005

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A 54-year-old woman was admitted to our hospital because of acute liver injury. Since she had a history of having used a diet product, drug-induced liver injury (DILI) was initially considered. However, the patient was subsequently diagnosed as suffering from primary biliary cirrhosis (PBC) based on the findings of liver histology and serum anti-mitochondrial antibody positivity. Overlap syndrome between PBC and autoimmune hepatitis (AIH) was also suspected, however, serum levels of aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase became normal three months later, after treatment with combination therapy comprising ursodeoxycholic acid plus bezafibrate. We therefore concluded that the liver disease in this patient was actually PBC, but that it resembled overlap syndrome or DILI. In cases of PBC, a rapid onset, as frequently seen in the case of DILI, viral hepatitis or AIH, is not common. We herein report a rare case of PBC which resembled DILI. (Internal Medicine 44: 1051-1054, 2005)

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Antimitochondrial antibody negative primary biliary cirrhosis: a distinct syndrome of autoimmune cholangitis.

Abstract: This study reports on a

Cited by 229 publications

References 30 publications

Autoimmune hepatitis with incidental histologic features of bile duct injury

Autoimmune hepatitis with incidental histologic features of bile duct injury

Management of primary biliary cirrhosis

Rapid-onset Primary Biliary Cirrhosis Resembling Drug-induced Liver Injury

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