Primary biliary cirrhosis with additional features of autoimmune hepatitis: Response to therapy with ursodeoxycholic acid

Joshi, Supriya; Cauch‐Dudek, Karen; Wanless, Ian R.; Lindor, Keith D.; Jorgensen, Roberta A.; Batts, Kenneth P.; Heathcote, E. Jenny

doi:10.1053/jhep.2002.30902

Cited by 170 publications

(130 citation statements)

References 25 publications

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“…Whereas PBC diagnosis is facilitated by the detection of antimitochondrial antibodies in the serum, that of PSC largely depends on cholangiographic evidence of multiple strictures of the biliary tract. [3][4][5][6][7][8] In children, it is known that AIH commonly exists concurrently with sclerosing cholangitis (SC). In a large pediatric series, half of the children with a definite diag-nosis of AIH demonstrated cholangiographic features of SC at presentation.…”

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confidence: 99%

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

et al. 2009

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The development of sclerosing cholangitis (SC) is observed in up to 50% of children followed up for autoimmune hepatitis (AIH). In adults, the prevalence is less known, although a recent study found evidence of SC in 10% of AIH patients using magnetic resonance cholangiopancreatography (MRCP). The aim of this study was to assess prospectively the prevalence of SC in adults with AIH. Fifty-nine consecutive patients with AIH diagnosed according to International Autoimmune Hepatitis Group score (women, 71%; mean age, 48 years; cirrhosis, 23%) underwent both MRCP and percutaneous liver biopsy. Twenty-seven patients with cirrhosis of nonbiliary or non-autoimmune etiology served as controls. Fourteen AIH patients (24%) showed mild MRCP abnormalities of intrahepatic bile ducts (IHBDs). None had abnormal common bile duct or convincing evidence of SC on MRCP or biopsy. A diagnosis of overlapping SC was nevertheless retained in one patient with MRCP abnormalities who subsequently developed symptomatic cholestasis despite corticosteroid therapy. Fibrosis score was the only independent parameter associated with bile duct abnormalities on MRCP (odds ratio 2.4; 95% confidence interval 1.4-4.7) and the percentage of patients with IHBD MRCP abnormalities was not different among F3-F4 AIH patients (n ‫؍‬ 24) and cirrhotic controls (46% versus 59%; NS). Conclusion: In this cohort of adult patients with AIH, the prevalence of SC was much lower than previously reported (1.7%). Mild MRCP abnormalities of IHBD were seen in a quarter of patients, but these abnormalities resulted from hepatic fibrosis and not SC. In the absence of cholestatic presentation, MRCP screening does not seem justified in adult-onset AIH. (HEPATOLOGY 2009;50:528-537.)

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confidence: 99%

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

et al. 2009

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“…Liver transplantation shows a 30% recurrence in transplants. 16 Our patient's pruritus improved with UDCA.…”

Section: Discussionmentioning

confidence: 66%

Primary Biliary Cirrhosis in a Male: A Case Report and Review of Literature

et al. 2015

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“…15 The short-term observation of these patients who where included in UDCA trials for PBC had similar improvement in biochemistries under UDCA in patients with or without features of PBC-AIH overlap syndrome. Consistent with this observation, two of our patients had spontaneously resolving flare-up of AIH and, at the time of this …”

Section: Discussionmentioning

confidence: 84%

“…This prevalence falls within the range (2%-20%) reported in case series published since 1998. [14][15][16][17][18] To date, consecutive forms of PBC-AIH overlap syndrome are thought to be rare. However, our data suggest that this variant is not uncommon, because 4% of our PBC patients developed superimposed AIH.…”

Section: Discussionmentioning

confidence: 99%

Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis

et al. 2006

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Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is a clinical entity characterized by the occurence of both conditions at the same time in the same patient. In addition to PBC-AIH overlap syndrome, transitions from one autoimmune disease to another have been reported, but no systematic series have been published. We report a series of 12 patients with consecutive occurrence of PBC and AIH (i.e., PBC followed by AIH). Among 282 PBC patients, 39 were identified who fulfilled criteria for probable or definitive AIH. AIH developed in 12 patients (4.3%). The baseline characteristics of the patients were similar to those of patients with classical PBC. Time elapsed between the diagnosis of PBC and the diagnosis of AIH varied from 6 months to 13 years. Patients with multiple flares of hepatitis at the time of diagnosis of AIH had cirrhosis on liver biopsy. Ten patients were given prednisone ؎ azathioprine; short-term as well as sustained remissions were obtained in 8 of these, while two had multiple relapses and eventually died 8 and 7 years after diagnosis of AIH. In conclusion, the development of superimposed AIH could not be predicted from baseline characteristics and initial response to UDCA therapy. If not detected early, superimposed AIH can result in rapid progression toward cirrhosis and liver failure in PBC patients. (HEPATOLOGY 2006;44:85-90.)

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Primary biliary cirrhosis with additional features of autoimmune hepatitis: Response to therapy with ursodeoxycholic acid

Cited by 170 publications

References 25 publications

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

Primary Biliary Cirrhosis in a Male: A Case Report and Review of Literature

Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis

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