Autoimmune Hepatitis Overlapping With Primary Sclerosing Cholangitis in Five Cases

McNair, Alistair N.B.; Moloney, Mairéad; Portmann, Bernard; Williams, Roger; McFarlane, Ian G.

doi:10.1111/j.1572-0241.1998.224_a.x

Cited by 147 publications

(64 citation statements)

References 26 publications

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“…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. Because our patients with and without biliary changes had comparable laboratory indices, similar histologic activity and fibrosis scores, comparable frequencies of cirrhosis, and adequate follow-up to discount the emergence of another disease, differences in severity, or the inadvertent inclusion in our analysis of transitional states are unlikely explanations for the findings.…”

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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“…These findings suggest that autoimmune cholangitis that is discovered prospectively may be a heterogeneous group that includes patients with atypical PBC, 52 small-duct PSC, 29,53 or a disease in transition. [54][55][56][57][58] Alternatively, it may be a separate disorder with variable histological manifestations that at times resembles PBC, PSC, or even AIH. Importantly, our patients with histological features of PBC or PSC were in the early stages of their disease, and histological changes could be discriminatory.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune cholangitis within the spectrum of autoimmune liver disease

et al. 2000

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“…20 Furthermore, another condition may be superimposed on the original process, such as PSC, [21][22][23][24] viral infection, 25 drug toxicity, 26 or fatty liver disease, 27 which could alter treatment outcome. Last, treatment failure may reflect intrinsic pathogenic mechanisms promoted by host-dependent genetic predispositions that cannot be suppressed by conventional corticosteroid regimens.…”

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confidence: 99%

Features associated with treatment failure in type 1 autoimmune hepatitis and predictive value of the model of end-stage liver disease

2007

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Autoimmune hepatitis may fail to respond to corticosteroid therapy, but the frequency and bases for this outcome are uncertain. We aimed to determine the frequency and nature of treatment failure in patients with type 1 autoimmune hepatitis, define features associated with its occurrence, and assess if the model for end-stage liver disease can predict this outcome. Patients failing conventional corticosteroid regimens were compared to patients who responded to similar regimens. Fourteen of 214 patients (7%) failed corticosteroid treatment. Patients who failed therapy were younger (33 ؎ 3 years versus 48 ؎ 1 years, P ‫؍‬ 0.0008), had higher serum levels of bilirubin at accession (4.1 ؎ 0.9 mg/dL versus 2.3 ؎ 0.2 mg/dL, P ‫؍‬ 0.02), presented acutely more frequently (43% versus 14%, P ‫؍‬ 0.01), and had a higher frequency of HLA (human leukocyte antigen) DRB1*03 (93% versus 53%, P ‫؍‬ 0.004) than did patients who achieved remission. An alternative disease (fatty liver disease) emerged in only 1 patient who failed therapy (7%). Scores determined by the model of end-stage liver disease at presentation of patients who failed treatment were higher than those of who achieved remission (16 ؎ 1 versus 10 ؎ 0.3 points, P < 0.0001), and score greater than 12 points had greater sensitivity (97%) and specificity (68%) for treatment failure than did HLA DRB1*03 or other features. Conclusion: Onset at an early age, acute presentation, hyperbilirubinemia, and presence of HLA DRB1*03 characterize patients who fail corticosteroid treatment. The model for end-stage liver disease may be a useful instrument for identifying patients prone to this outcome. (HEPATOLOGY 2007;46:1138-1145

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Autoimmune Hepatitis Overlapping With Primary Sclerosing Cholangitis in Five Cases

Cited by 147 publications

References 26 publications

Autoimmune hepatitis with incidental histologic features of bile duct injury

Autoimmune hepatitis with incidental histologic features of bile duct injury

Autoimmune cholangitis within the spectrum of autoimmune liver disease

Features associated with treatment failure in type 1 autoimmune hepatitis and predictive value of the model of end-stage liver disease

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