Diagnosis and treatment of autoimmune hepatitis

Czaja, Albert J.; Freese, Deborah K.

doi:10.1053/jhep.2002.34944

Cited by 524 publications

(478 citation statements)

References 203 publications

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“…in accordance with a previously published clinical protocol constituted the study population. 23 All patients had been referred to the Autoimmune Hepatitis Treatment Program of the Mayo Clinic, and they had been evaluated between 1975 and 2005. Study patients were selected from 302 patients who had been seen during this same interval because they satisfied the following additional criteria.…”

Section: Methodsmentioning

confidence: 99%

“…26 Liver tissue examinations were performed at presentation in 203 of the 205 patients (99%), and the histological findings of autoimmune hepatitis were documented in each, including 53 patients (26%) who satisfied histological criteria for cirrhosis. 27 Concurrent immune diseases were sought systematically in each patient according to protocol, 23 and HLA DR3 and DR4 were determined in each individual by restriction fragment length polymorphism (84 patients) or polymerase chain reaction with sequence specific primers (121 patients) according to methods reported previously. 17,18 Our study had been approved by the Institutional Review Board of the Mayo Clinic.…”

Section: Methodsmentioning

confidence: 99%

“…One hundred seventy-nine patients (87%) were treated with conventional corticosteroid regimens, and the treatment outcomes between the patients aged Ն60 years and those aged Յ30 years were compared. 23 One hundred fifteen patients received prednisone in combination with azathioprine (64%), and 64 patients received a higher dose of prednisone alone (36%). Each schedule had been shown previously to be equally effective in treating autoimmune hepatitis.…”

Section: Methodsmentioning

confidence: 99%

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Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly

2006

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Autoimmune hepatitis is classically a disease of young women. Our aims were to determine its occurrence, clinical phenotype, and outcome in elderly patients and contrast findings to young adults. Two-hundred-and-five white North American adults with definite type 1 autoimmune hepatitis were grouped according to age at presentation and the groups compared. Forty-seven patients (23%) were aged >60 years (median age, 68 years), and 31 patients (15%) were aged <30 years (median age, 25 years). The patients >60 years had a higher frequency of cirrhosis at presentation than the patients <30 years (33% versus 10%, P ‫؍‬ .03). They also had thyroid or rheumatic diseases more commonly (42% vs. 13%, P ‫؍‬ .006). HLA DR3 occurred more frequently in the patients <30 years than in those >60 years (58% vs. 23%, P ‫؍‬ .004), and HLA DR4 occurred more often in the patients >60 years (47% vs. 13%, P ‫؍‬ .003). Patients aged >60 years failed corticosteroid treatment less commonly than those aged <30 years (5% vs. 24%, P ‫؍‬ .03). Autoimmune hepatitis occurred in patients aged 18-30 years (15%), 31-39 years (15%), 40-49 years (21%), 50-59 years (25%), and >60 years (23%). Differences in age distribution, HLA frequencies, and treatment outcome occurred after age >40 years. In conclusion, elderly patients have a greater frequency of cirrhosis at presentation and HLA DR4 than patients <30 years, and they have a lower occurrence of treatment failure. Transitions in clinical and genetic phenotypes occur after age >40 years. Genetic susceptibilities may favor etiologic factors that are age-related. (HEPATOLOGY 2006;43:532-538.)

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly

2006

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“…Each had been treated with conventional corticosteroid regimens, had fulfilled preestablished clinical, laboratory, and histological criteria for having experienced either treatment failure or remission, and had been systematically monitored according to a previously published protocol. 42 One hundred seventy-four patients (81%) were female, and the mean age at presentation was 47 Ϯ 1 years (median, 49 years; range, 13-82 years). One hundred forty-seven patients (69%) had been treated with prednisone in combination with azathioprine, and 67 patients (31%) had received a higher dose of prednisone alone.…”

Section: Methodsmentioning

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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“…Non-organ-specific autoantibodies, a requisite for diagnosis, typically include anti-smooth muscle antibodies and antinuclear antibodies, as well as antibodies to liver kidney microsome type 1. 71 Their occurrence implies activation of immune mechanisms possibly involved primarily in disease pathogenesis or collateral responses to liver cell destruction and nonselective antigen release. Autoantibodies after liver transplantation do not establish the diagnosis of AIH, nor are they accurate parameters of inflammatory activity.…”

Section: Recurrent Diseases and New-onset Diseasesmentioning

confidence: 99%

Liver biopsy interpretation for causes of late liver allograft dysfunction

2006

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Evaluation of needle biopsies and extensive clinicopathological correlation play an important role in the determination of liver allograft dysfunction occurring more than 1 year after transplantation. Interpretation of these biopsies can be quite difficult because of the high incidence of recurrent diseases that show histopathological, clinical, and serological features that overlap with each other and with rejection. Also, more than one insult can contribute to allograft injury. In an attempt to enable centers to compare and pool results, improve therapy, and better understand pathophysiological disease mechanisms, the Banff Working Group on Liver Allograft Pathology herein proposes a set of consensus criteria for the most common and problematic causes of late liver allograft dysfunction, including late-onset acute and chronic rejection, recurrent and new-onset viral and autoimmune hepatitis, biliary strictures, and recurrent primary biliary cirrhosis and primary sclerosing cholangitis. A discussion of differential diagnosis is also presented. (HEPATOLOGY 2006;44:489-501.)

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Diagnosis and treatment of autoimmune hepatitis

Cited by 524 publications

References 203 publications

Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly

Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly

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

Liver biopsy interpretation for causes of late liver allograft dysfunction

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