Simplified Scoring for AIH
Formula :
Composite score of listed variables. Points given for each variable.
Explanation of Result :
> 6 is consistent with definite diagnosis. 6 is consistent with probable diagnosis.
* Please verify all calculations prior to clinical use.
Reference(s) :
- PMID: 19402135.
- Yeoman AD, Westbrook RH, Al-Chalabi T, Carey I, Heaton ND, Portmann BC, Heneghan MA. Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group (IAIHG) criteria in acute and chronic liver disease. Hepatology. 2009 Aug;50(2):538-45. PMID: 19575457.
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 >or=6 (probable AIH) and >or=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 >or=6, sensitivity was 90%, and specificity was 98% with positive and negative predictive values of 97% and 92%, respectively. For scores >or=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 >or=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.
- Fujiwara K, Yasui S, Tawada A, Fukuda Y, Nakano M, Yokosuka O. Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group criteria in acute-onset autoimmune hepatitis. Liver Int. 2011 Aug;31(7):1013-20. PMID: 21733091.
Abstract
BACKGROUND:
The diagnosis of autoimmune hepatitis (AIH) is already difficult, and that of acute-onset AIH with atypical features is even more challenging, even though the revised original diagnostic criteria created by an international AIH group were widely accepted and incorporated into clinical practice.
AIMS:
Recently, simplified diagnostic criteria were proposed. We compared the performance parameters of the simplified scoring system in patients with acute-onset AIH and examined its usefulness and limitations.
METHODS:
Fifty-five patients with acute-onset AIH (29 non-severe, 14 severe and 12 fulminant) were assessed according to the simplified scoring system and compared with the revised original one.
RESULTS:
Of the 55 patients, 22 (40%) were diagnosed as ‘definite’ AIH, 28 (51%) as ‘probable’ and five (9%) as ‘non-diagnostic’ based on the revised original scoring system. By the simplified scoring system, six (11%) were diagnosed as ‘definite’ AIH, 16 (29%) as ‘probable’ and 33 (60%) as ‘non-diagnostic’. Anti-nuclear antibody titres did not differ among the three groups. The immunoglobulin G level was higher in fulminant than in non-severe patients (P = 0.01). Sixty-five per cent showed acute hepatitis (massive necrosis, submassive necrosis and severe acute hepatitis) and 35% showed chronic hepatitis.
CONCLUSIONS:
The revised original scoring system performed better in patients with acute-onset AIH than the simplified scoring system.
- Mileti E, Rosenthal P, Peters MG. Validation and modification of simplified diagnostic criteria for autoimmune hepatitis in children. Clin Gastroenterol Hepatol. 2012 Apr;10(4):417-21. PMID: 22179022.
Abstract
BACKGROUND & AIMS:
Criteria for the diagnosis of autoimmune hepatitis (AIH) were formalized in 1993 and revised in 1999. Simplified criteria were developed in 2008 for adults only. We aimed to establish clinically useful diagnostic criteria for AIH in children by validating the 2008 criteria in a pediatric cohort.
METHODS:
Baseline data were available in 37 and 31 AIH and 40 and 26 non-AIH subjects to calculate 1999 and 2008 criteria, respectively. Sensitivity and specificity of the simplified criteria were calculated using 1999 criteria as the standard for subjects with available data for both criteria.
RESULTS:
The 1999 standard designated 29 of 31 subjects (94%) as definite AIH and 2 of 31 subjects (6%) as probable AIH. The simplified criteria identified 25 of 31 subjects (81%) as definite AIH, 2 of 31 subjects (6%) as probable AIH. Only 1 of 5 patients with AIH who presented with fulminant hepatic failure (FHF) was identified by the simplified criteria as having AIH. The 2008 diagnostic criteria had a sensitivity of 87% and a specificity of 89% (area under the receiver operating characteristic curve, 0.98). After removing data from patients with FHF from the analysis, the sensitivity increased to 100%. Modifying the 2008 diagnostic criteria to include either level of globulin or immunoglobulin G resulted in a similar sensitivity (92%) and specificity (95%) values (area under the receiver operating characteristic curve, 0.99).
CONCLUSIONS:
The 2008 criteria diagnose AIH in children with high levels of sensitivity and specificity, and are easier to use in the clinic. Diagnosis of AIH in patients who present in FHF requires the 1999 criteria. Levels of globulin and immunoglobulin G can be used interchangeably in the simplified diagnostic criteria.