Evolution of autoimmune hepatitis to primary sclerosing cholangitis: A sequential syndrome

Abdo, Ayman A.; Bain, Vincent G.; Kichian, Krikor; Lee, Samuel S.

doi:10.1053/jhep.2002.37200

Cited by 73 publications

(34 citation statements)

References 24 publications

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“…In adults, systematic investigations of AIH-SC overlap are scarce. Abdo et al 6 described 6 adult patients diagnosed with SC many years after well-established AIH. This overlap was identified only when severe cholestasis and/or resistance to standard immunosuppressive therapy was observed a mean of 4.6 years following the initial diagnosis and initiation of treatment for AIH.…”

Section: Discussionmentioning

confidence: 99%

“…Subsequently, Gregorio et al 5 made a prospective study of a large series of children with clinically overt AIH and found that 27 of 55 (49%), all of whom underwent ERCP at the time of initial diagnosis of AIH, had cholangiographic evidence of what the authors termed autoimmune sclerosing cholangitis. The only case series in adults was reported by Abdo et al, 6 who described 6 adults initially given a diagnosis of AIH, 3 of whom had undergone ERCP at initial presentation. All 6 had no histological evidence of duct involvement on their baseline liver biopsy.…”

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confidence: 99%

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Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: Evaluating the role of routine magnetic resonance imaging

et al. 2007

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Large bile duct injury (that seen on cholangiography) is not usually considered a feature of autoimmune hepatitis (AIH) in adults but is present in up to 50% of children with AIH. The aim of this work was to study the prevalence of large bile duct abnormalities identified by magnetic resonance cholangiography (MRC) in adults given a diagnosis of AIH. Seventy-nine (n ‫؍‬ 79) patients given a diagnosis of AIH (mean AIH score: 15.1 ؎ 3.4) were screened with MRC for evidence of sclerosing cholangitis (SC). Results were reviewed by two radiologists. Clinical parameters were correlated with MRC findings. A histological review of available liver biopsies (n ‫؍‬ 29) was performed. Of the 79 patients surveyed, 8 (10%) had MRC findings consistent with primary sclerosing cholangitis (PSC). The interrater variability was excellent (kappa ‫؍‬ 0.87). Younger age at diagnosis (24.3 ؎ 11.9), higher baseline alkaline phosphatase (186.4 ؎ 98.3), higher bilirubin at time of MRC (45.8 ؎ 37.2), and greater lobular activity on initial liver biopsy were significantly associated with the detection of this overlap of SC with AIH (P ‫؍‬ 0.024, P ‫؍‬ 0.037, P ‫؍‬ 0.032, and P ‫؍‬ 0.041, respectively), but not alkaline phosphatase/aspartate aminotransferase ratio, time between the initial diagnosis of AIH and the MRC, or the presence of cirrhosis on initial liver histology. Two cases with a normal MRC had histological lesions typical of small duct PSC. Conclusion: The presence of SC detected by MRC and from liver histology in adult patients with AIH may not be clinically overt, and thus the prevalence of this AIH/SC overlap may be higher than previously recognized. Our data suggest that routine radiological evaluation of the biliary tree should be performed in adults given a diagnosis of AIH, as in children the presence of this overlap negatively impacts on survival. (HEPATOLOGY 2008;47:949-957.)

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: Evaluating the role of routine magnetic resonance imaging

et al. 2007

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“…9 Sequential evolution of AIH toward SC has also been reported in children and young adults, mainly those who became resistant to immunosuppressive treatment or developed cholestasis. 9,10 This so-called autoimmune sclerosing cholangitis affects boys and girls equally and is more likely to progress to cirrhosis, liver transplantation, or death rather than AIH. 11 In marked contrast, the prevalence of underlying SC in adult-onset AIH is poorly understood.…”

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confidence: 99%

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: A prospective magnetic resonance imaging and histological study

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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“…Both AIH and PSC can be associated with inflammatory bowel disease (IBD) [40,48], and there is evidence that they can occur sequentially in patients who present with typical AIH subsequently developing PSC [43,[49][50][51]. Evidence from our laboratory suggests that the liver disease is driven by the recruitment of effector lymphocytes that were activated in the gut [52].…”

Section: Overlap Syndromes With Other Autoimmune Liver Diseasesmentioning

confidence: 99%

“…They usually occur at the same time (true overlap syndromes), but in some cases, patients present with features of one disease and subsequently develop features of the other, sometimes after intervals of several years (sequential overlap syndromes). Sequential syndromes involving PBC and AIH typically present with features of PBC first, whereas in those involving PSC and AIH, features of AIH usually occur first [43].…”

Section: Overlap Syndromes With Other Autoimmune Liver Diseasesmentioning

confidence: 99%

Autoimmune hepatitis: new paradigms in the pathogenesis, diagnosis, and management

2010

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Autoimmune hepatitis (AIH), primary biliary cirrhosis, and primary sclerosing cholangitis are the three major autoimmune diseases affecting the liver, and of these three, AIH is the most typical autoimmune disease being characterized by a T-cell-rich infiltrate, raised circulating c-globulins, autoantibodies, HLA associations, and links with other autoimmune diseases. It is the only one, of the three diseases, that responds well to immunosuppressive therapy. AIH is caused by dysregulation of immunoregulatory networks and the consequent emergence of autoreactive T cells that orchestrate a progressive destruction of hepatocytes leading untreated to liver failure. T cells play a major role in the immunopathogenesis, and both CD4? and CD8? T cells are involved together with effector responses mediated by NK cells, cd T cells, and macrophages. A number of triggering factors have been proposed including viruses, xenobiotics, and drugs, but none have been conclusively shown to be involved in pathogenesis.

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Evolution of autoimmune hepatitis to primary sclerosing cholangitis: A sequential syndrome

Cited by 73 publications

References 24 publications

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: Evaluating the role of routine magnetic resonance imaging

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: Evaluating the role of routine magnetic resonance imaging

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: A prospective magnetic resonance imaging and histological study

Autoimmune hepatitis: new paradigms in the pathogenesis, diagnosis, and management

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