Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group (IAIHG) criteria in acute and chronic liver disease†

Abstract

Diagnostic criteria for autoimmune hepatitis (AIH) have been created and revised by the International Autoimmune Hepatitis Group (IAIHG). Simplified criteria have been created, but remain independently unvalidated. We report on the diagnostic accuracy of the simplified criteria in patients across a range of diagnoses, including a subset of patients presenting with fulminant liver failure who required liver transplant. Patients with AIH and non-AIH etiologies of liver disease were identified from dedicated patient databases. Parameters relevant to the simplified and 1999 IAIHG criteria were recorded, and sensitivity, specificity, and positive and negative predictive values for scores of >6 (probable AIH) and >7 (definite AIH) were calculated. A total of 549 patients with chronic liver disease were evaluated, (221 with AIH, 26 with variant syndromes, and 302 with non-AIH). For scores >6, sensitivity was 90%, and specificity was 98% with positive and negative predictive values of 97% and 92%, respectively. For scores >7; sensitivity was 70%, and specificity was 100% with positive and negative predictive values of 100% and 74%, respectively. Seven false positive diagnoses of AIH occurred, all with simplified scores of 6. Concordance with 1999 criteria was 90% for probable and 61% for definite AIH. The frequency of an overall diagnosis of AIH (probable or definite AIH) among the 70 patients with fulminant liver failure was 24% for simplified criteria and 40% for 1999 criteria, respectively. Conclusion: The simplified criteria retain high specificity but exhibit lower sensitivity for scores of >7. The explanations for this are unclear but may relate to loss of such discriminating information as response to corticosteroids. Prospective evaluation of these criteria is required to corroborate these observations. (HEPATOLOGY 2009;50:538-545.)

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

“…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%. The improved diagnosis of acute autoimmune hepatitis would facilitate timely immunosuppressive therapy as the high necroinflammatory activity in these cases can potentially lead to rapid progression of fibrosis if left untreated.…”

“…2 The Revised criteria comprise 12 parameters, and are too complex for routine use. [3][4][5] In addition to being cumbersome, these criteria require serologic tests that are expensive and not widely available. 3 In 2008, the International Autoimmune Hepatitis Group proposed simplified criteria that incorporate four parameters: 3 autoantibodies, serum immunoglobulin G, liver biopsy findings, and results of viral hepatitis work-up (Table 1).…”

“…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.…”

“…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).…”

Direct acting antiviral therapy is curative for chronic hepatitis C/autoimmune hepatitis overlap syndrome

“…To standardize diagnostic criteria, the International Autoimmune Hepatitis Group (IAHG) devised a scoring system to categorize patients as definite AIH, probable AIH and not AIH [2] in which points are distributed based on the presence of antinuclear antibody (ANA), anti smooth antibody (ASMA or F-Actin Antibody), anti-soluble liver/liver pancreas antigen, immunoglobulin G (IgG) level, liver histology and the absence of viral hepatitis. In patients with chronic hepatitis C, markers of AIH are frequently present.…”