Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group criteria in acute-onset autoimmune hepatitis

Fujiwara, Keiichi; Yasui, Shin; Tawada, Akinobu; Fukuda, Yoshihiro; Nakano, Masayuki; Yokosuka, Osamu

doi:10.1111/j.1478-3231.2011.02524.x

Cited by 69 publications

(77 citation statements)

References 25 publications

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“…A histologic score of 2 was not observed in any acute autoimmune hepatitis case in our study, which is similar to the 3.6% figure reported earlier. 8 In these cases, the reported sensitivity of simplified score for probable or definite autoimmune hepatitis is 0-28%. 5,7,8 When the modified histologic criteria were used, histologic score of 2 was observed in 27 (93%) of our cases, and the sensitivity for probable/definite autoimmune hepatitis went up from 62 to 90%.…”

Section: Discussionmentioning

confidence: 99%

“…The penetration of lymphocytes and/or plasma cells into hepatocytes has an important role inflicting hepatocellular injury as part of the pathogenesis of autoimmune hepatitis, but its determination by light microscopy is challenging. 8 Most studies that evaluate emperipolesis do not provide any definite criteria for evaluation of this feature, 13 but clear criteria were used in one study. 14 We used similar criteria, scoring this feature as present only when intact lymphocytes or plasma cells appeared to be in the cytoplasm of hepatocytes, while cytoplasmic nuclear fragments were not considered.…”

Section: Discussionmentioning

confidence: 99%

“…7,13,14,16 The presence of emperipolesis is best evaluated by confocal or electron microscopy and it is difficult to reliably assess this feature in routine diagnostic practice with light microscopy. 2,8,15 In our experience, both these features are rarely mentioned in pathology reports in the United States, even in large specialized academic centers. This suggests that most cases of otherwise characteristic autoimmune hepatitis would only get a histologic score of 1 point in the absence of rosettes and emperipolesis, as both these features are required along with interface activity for a histologic score of 2 points.…”

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confidence: 97%

“…3,5 The sensitivity for diagnosis of autoimmune hepatitis is somewhat limited, with reported numbers ranging from 65 to 95% for probable autoimmune hepatitis (score 46) and from 15 to 87% for definite autoimmune hepatitis (score 47). 3,4,6 The International Autoimmune Hepatitis Group criteria do not perform well in making the diagnosis of autoimmune hepatitis in specific situations such as cases with acute presentation, 5,7,8 overlap syndromes, 5,9,10 and in the setting of concurrent diseases like nonalcoholic fatty liver disease. 11 Autoimmune hepatitis with acute presentation clinically present as acute hepatitis; a subset of these cases show histologic features of chronicity such as fibrosis on biopsy, while others resemble acute hepatitis on histology as well.…”

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confidence: 99%

“…11 Autoimmune hepatitis with acute presentation clinically present as acute hepatitis; a subset of these cases show histologic features of chronicity such as fibrosis on biopsy, while others resemble acute hepatitis on histology as well. 5,7,8,12 Histologic features have a critical role in the diagnosis of autoimmune hepatitis, and identification of a hepatitic pattern of injury is considered a pre-requisite for applying the simplified criteria. 3 As per the simplified criteria, the biopsy findings are considered typical (2 points), compatible (1 point), and atypical (0 points).…”

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confidence: 99%

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Autoimmune hepatitis: review of histologic features included in the simplified criteria proposed by the international autoimmune hepatitis group and proposal for new histologic criteria

et al. 2017

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Simplified criteria for diagnosis of autoimmune hepatitis are based on autoantibodies, serum immunoglobulin G, histologic features, and negative viral serology. A score of 6 points is necessary for the designation of probable autoimmune hepatitis and 7 points or more for definite autoimmune hepatitis. The presence of three histologic features is required for categorizing a case as typical (2 points): interface hepatitis with portal lymphocytic/ lymphoplasmacytic cells extending into lobule, emperipolesis, and rosettes. In the absence of all three features, a chronic hepatitis picture is considered compatible with autoimmune hepatitis (1 point). This study examines the validity of these histologic features for the diagnosis of autoimmune hepatitis. Clinical data and liver biopsies were reviewed for 88 autoimmune hepatitis, 20 primary biliary cholangitis, and 13 non-autoimmune acute hepatitis cases. Interface/lobular activity, number of plasma cells, copper/CK7 stains, and presence/absence of biliary features were assessed in autoimmune hepatitis and primary biliary cholangitis cases. The simplified criteria score was calculated. Modified histologic criteria were formulated on the basis of interface/lobular activity, number of plasma cells, and presence/absence of biliary features. Using the proposed histologic features, histologic score of 2 increased from 8 to 77%, while total simplified score of 46 increased from 69 to 86%. There was no increase in total simplified score for primary biliary cholangitis or non-autoimmune acute hepatitis. Rosettes and emperipolesis are difficult to interpret, and lack sensitivity and sensitivity for autoimmune hepatitis diagnosis. The current histologic criteria used in the current simplified score lead to underscoring of autoimmune hepatitis cases. The modified histologic criteria based on the inflammatory activity, extent of plasma cells, and results of copper/CK7 staining increased the histologic score in autoimmune hepatitis and led to a probable/definite diagnosis of autoimmune hepatitis in 17% of cases that would have otherwise been classified as non-autoimmune hepatitis by simplified score.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 97%

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confidence: 99%

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confidence: 99%

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Autoimmune hepatitis: review of histologic features included in the simplified criteria proposed by the international autoimmune hepatitis group and proposal for new histologic criteria

et al. 2017

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Histological Discrimination Between Autoimmune Hepatitis and Drug-Induced Liver Injury

Fujiwara

Yokosuka

2012

Hepatology

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We read with interest the article by Khalili et al. 1 addressing the management of small liver nodules detected in patients with cirrhosis under surveillance with abdominal ultrasound (US) that gave indeterminate results by contrast imaging. To optimize American Association for the Study of the Liver Disease (AASLD) guidelines, 2 the authors suggest performing a fine-needle biopsy examination of nodules showing either arterial hypervascularity on computed tomography (CT) / magnetic resonance imaging (MRI) or accompanied by a synchronous hepatocellular carcinoma (HCC) only, since these were the only independent variables associated with malignancy in their retrospective study. According to this algorithm, approximately 20% of additional tumors will be identified, with a sensitivity of 62%, a specificity of 79%, and a 73% save of liver biopsies.When we applied this algorithm to our patients with a de novo liver nodule prospectively detected during surveillance 3 ( Fig. 1), the corresponding figures were 44% for sensitivity and 55% for specificity, with a positive predictive value of 44% and a negative predictive value of 55%, respectively. Overall, among 36 1-2 cm indeterminate nodules the modified algorithm would have diagnosed 7 (44%) of tumors only of the 16 identified by histology, including 15 HCC and 1 intrahepatic cholangiocarcinoma (ICC). At the same time, the diagnosis of HCC would have been significantly delayed in nine (56%) patients compared with none if treated according to AASLD guidelines. The fact that the majority (75%) of delayed diagnoses were in patients with a very early HCC, i.e., the ideal candidates for radical treatment with local ablation, 4 attenuates the appeal of the modified algorithm, which in addition would have also led to a misdiagnosis of ICC in one nodule devoid of contrast uptake during the arterial phase of CT/MRI. Due to the high incidence of HCC in patients with compensated cirrhosis and the low risk of liver biopsy complications, we strongly endorse unmodified AASLD guidelines for the management of patients with cirrhosis with 1-2 cm liver nodules with undefined radiological diagnosis. Fig. 1. The diagnostic yield of revised AASLD algorithm in our patients undergoing surveillance. Liver histology was the gold standard for the diagnosis of 1-2 cm HCC. 651Reply:We thank Drs. Iavarone and Sangiovanni for the interest in our study, 1 but caution the authors with regard to several points in their letter. First, their conclusions are based on a small sample size of 36 indeterminate nodules. While they calculate sensitivity and specificity of 44% and 55%, respectively, using our proposed criteria, the 95% confidence interval was not reported. We calculate their confidence interval to be 21%-69% for sensitivity and 32%-76% for specificity. Small sample sizes lead to real uncertainty.Second The substantial lower sensitivity reported by Iavarone and Sangiovanni may be a result of their small sample size, but should lead to reexamination of their methodology. It is unclear whether ...

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High level of persistent liver injury is one of clinical characteristics in treatment‐naïve acute onset autoimmune hepatitis: experience in a community hospital

Fujiwara

Fukuda

Seza

et al. 2016

J Hepato Biliary Pancreat

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Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group criteria in acute-onset autoimmune hepatitis

Abstract: The revised original scoring system performed better in patients with acute-onset AIH than the simplified scoring system.

Cited by 69 publications

References 25 publications

Autoimmune hepatitis: review of histologic features included in the simplified criteria proposed by the international autoimmune hepatitis group and proposal for new histologic criteria

Autoimmune hepatitis: review of histologic features included in the simplified criteria proposed by the international autoimmune hepatitis group and proposal for new histologic criteria

Histological Discrimination Between Autoimmune Hepatitis and Drug-Induced Liver Injury

High level of persistent liver injury is one of clinical characteristics in treatment‐naïve acute onset autoimmune hepatitis: experience in a community hospital

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