Revised Original Score for Autoimmune Hepatitis (AIH) Calculator
Diagnoses AIH in patients based on the revised scoring system of the original diagnostic criteria.
Refer to the text below the score for more information on its variables and result interpretation.
The revised AIH scoring system assists in diagnosing patients with confirmed hepatitis and liver biopsy results that show histological evidence. It simplifies the typically complex process of diagnosing AIH, which is challenging due to the lack of a definitive anatomical gold standard and the multifactorial, competing factors involved in AIH diagnoses.
| Revised AIH Score | Pre- or post treatment | Diagnosis |
| >15 | Pre-treatment | Definite AIH |
| 10 – 15 | Probable AIH | |
| <10 | Possible AIH – other testing recommended | |
| >17 | Post-treatment | Definite AIH |
| 12 – 17 | Probable AIH | |
| <12 | Possible AIH – other testing recommended |
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Steps on how to print your input & results:
1. Fill in the calculator/tool with your values and/or your answer choices and press Calculate.
2. Then you can click on the Print button to open a PDF in a separate window with the inputs and results. You can further save the PDF or print it.
Please note that once you have closed the PDF you need to click on the Calculate button before you try opening it again, otherwise the input and/or results may not appear in the pdf.
Items and interpretation
The revised AIH scoring system aids in diagnosing patients with confirmed hepatitis and liver biopsy findings showing histological evidence. It helps streamline the often complex AIH diagnosis process, which is complicated by the absence of a definitive anatomical gold standard and the multifactorial, competing nature of AIH diagnoses.
The table below summarises the items of the score and their associated number of points.
| Revised AIH Score Items | Points | |
| Sex | Male | 0 |
| Female | 2 | |
| ALP:AST or ALP:ALT ratio | <1.5 | 2 |
| 1.5 - 3.0 | 0 | |
| >3.0 | -3 | |
| Serum globulins or IgG, times above upper limit of normal | >2.0 | 3 |
| 1.5 - 2.0 | 2 | |
| 1.0 - 1.5 | 1 | |
| <1.0 | 0 | |
| Antinuclear antibody (ANA), smooth muscle antibody (SMA), or liver/kidney microsomal type 1 (LKM-1) antibody | >1:80 | 3 |
| 1:80 | 2 | |
| 1:40 | 1 | |
| <1:40 | 0 | |
| Hepatitis viral markers | Positive | -3 |
| Negative | 3 | |
| Recent or current use of hepatotoxic drugs | Yes | -4 |
| No | 1 | |
| Average alcohol intake | <25 g/day | 2 |
| 25-60 g/day | 0 | |
| >60 g/day | -2 | |
| Liver histology | Interface hepatitis | 3 |
| Predominantly lymphoplasmacytic infiltrate | 1 | |
| Rosetting of liver cells | 1 | |
| No interface hepatitis, not predominantly lymphoplasmacytic infiltrate, and no rosetting of liver cells | -5 | |
| Biliary changes (bile duct changes typical of primary biliary cirrhosis or primary scleroisng cholangitis, and/or periportal ductal reaction with copper-associated protein accumulation) | -3 | |
| Other changes (any other prominent features suggesting different etiology) | -3 | |
| Other autoimmune disease(s) (in patient or 1st degree relatives) | No | 0 |
| Yes | 2 | |
| Optional additional parameters (Assign points only in patients seronegative for ANA, SMA and LKM-1) |
Seropositivity for other defined autoantibodies (e.g. pANCA, anti-LCl, anti-SLA, anti-ASGPR, anti-LP and anti-sulfatide) |
2 |
| HLA-DR3 or HLA-DR4 | 1 | |
| Response to therapy | Complete | 2 |
| Relapse | 3 | |
| Anti-mitochondrial antibody (AMA) | Positive | -4 |
| Negative | 0 | |
Following the addition of the selected points, the final score is associated with a likelihood of autoimmune hepatitis diagnosis, with different cut-offs for pre and post-treatment.
| Revised AIH Score | Pre- or post treatment | Diagnosis |
| >15 | Pre-treatment | Definite AIH |
| 10 – 15 | Probable AIH | |
| <10 | Possible AIH – other testing recommended | |
| >17 | Post-treatment | Definite AIH |
| 12 – 17 | Probable AIH | |
| <12 | Possible AIH – other testing recommended |
In a comparison with the simplified scoring for AIH, the revised original scoring system had greater sensitivity for the diagnosis than the simplified scoring system (100% versus 95%), and seven patients diagnosed as AIH using the revised original system were nondiagnostic by the simplified system (5%).
On the other hand, the simplified diagnostic system had greater specificity (90% versus 73%) and predictability (92% versus 82%) for AIH than the revised original system, and it more commonly excluded the diagnosis in other diseases with concurrent immune features (83% versus 64%).
About the original study and further validation
This AIH revised scoring system has been devised following a review of the original diagnostic criteria by the International Autoimmune Hepatitis Group (IAIHG). The principal changes in the assigned scores relate to the ALP:AST (or ALT) ratio, drug history, liver histology and response to therapy.
Subsequent validation has shown that the revised original scoring system performs better in patients with few or atypical features of AIH, and the simplified system is better at excluding the diagnosis in diseases with concurrent immune manifestations.
These diagnostic scoring systems are valuable tools for diagnosing AIH and provide complementary benefits. However, they should not replace the gold standard of thorough clinical evaluation and are primarily useful for defining patient cohorts in research settings.
References
Original reference
Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, Cooksley WG, Czaja AJ, Desmet VJ, Donaldson PT, Eddleston AL, Fainboim L, Heathcote J, Homberg JC, Hoofnagle JH, Kakumu S, Krawitt EL, Mackay IR, MacSween RN, Maddrey WC, Manns MP, McFarlane IG, Meyer zum Büschenfelde KH, Zeniya M, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999; 31(5):929-38.
Validation
Czaja AJ. Performance parameters of the diagnostic scoring systems for autoimmune hepatitis. Hepatology. 2008; 48(5):1540-8.
Other references
Ducazu O, Degroote H, Geerts A, Hoorens A, Schouten J, Van Vlierberghe H, Verhelst X. Diagnostic and prognostic scoring systems for autoimmune hepatitis: a review. Acta Gastroenterol Belg. 2021; 84(3):487-495.
Specialty: Hepatology
Objective: Diagnosis
No. Of Items: 15
Year Of Study: 1999
Article By: Denise Nedea
Published On: October 4, 2024
Last Checked: October 4, 2024
Next Review: October 4, 2029