Autoimmune hepatitis laboratory findings

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: :Manpreet Kaur, MD [2]

Overview

Laboratory findings consistent with the diagnosis of autoimmune hepatitis include, increased serum aminotransferase levels (1.5-50 times reference values), Increased serum immunoglobulin levels, primarily immunoglobulin G (1.2 to 3.0 times normal) and seropositive for ANAs, anti-actin, SMAs, LKM-1, anti-liver cytosol 1 (anti-LC1) antibodies, SLA/LP autoantibodies or pANCA.

Laboratory Findings

Following laboratory tests are used to diagnose autoimmune hepatitis:

Liver function tests
Alkaline phosphatase
Antibody assays
Serum protein and immunoglobulins
Complete blood count and differential count
Platelet count
ESR
Coombs test
Peripheral blood film

Laboratory findings consistent with the diagnosis of autoimmune hepatitis include:

Increased serum aminotransferase levels (1.5-50 times reference values)
Increased serum immunoglobulin levels, primarily immunoglobulin G (1.2 to 3.0 times normal)
Seropositive for ANAs, anti-actin, SMAs, LKM-1, anti-liver cytosol 1 (anti-LC1) antibodies, SLA/LP autoantibodies or pANCA

Revised Scoring System of the International Autoimmune Hepatitis Group
Laboratory Findings	Points
AP:AST (or ALT) ratio <1.5 1.5-3 >3	+2 0 -2
Serum globulin or IgG level above ULN >2.0 1.5-2.0 1.5-1 <1	+3 +2 +1 0
ANA, SMA, or anti-LKM1 >1:80 1:80 1:40 <1:40	+3 +2 +1 0
AMA positive	-4
Hepatitis markers Positive Negative	-3 +3
Hepatotoxic drug exposure Positive Negative	-4 +1
Average alcohol intake (g/day) <25 >60	+2 -2
Histologic findings Interface hepatitis Lymphoplasmacytic infiltrate Rosette formation Biliary changes Other atypical changes None of above	+3 +1 +1 -3 -3 -5
Concurrent immune disease, including celiac disease	+2
Other autoantibodies	+2
HLA DRB103 or DRB104	+1
Response to corticosteroids Complete Relapse after drug withdrawal	+2 +3
Aggregate score pretreatment Definite autoimmune hepatitis – >15 Probable autoimmune hepatitis -10 -15 Aggregate score post-treatment Definite autoimmune hepatitis – >17 Probable autoimmune hepatitis- 12-17
AP, alkaline phosphatase; AST, aspartate aminotransferase; ALT, alanine aminotransferase; IgG, immunoglobulin G; ULN-upper limit of the normal range; HLA, human leukocyte antigen; ANA, antinuclear antibodies; SMA, smooth muscle antibodies; anti-LKM1, antibodies to liver kidney microsome type 1; AMA, antimitochondrial antibodies. Adapted from Alvarez F, et al. J Hepatol 1999;31:929–938, with permission of Elsevier BV and the European Association for the Study of the Liver.^[1]^[2]^[3]

Diagnostic features of the overlap syndromes of autoimmune hepatitis :^[4]

Diagnostic features of the overlap syndromes of autoimmune hepatitis
Type of overlap syndrome	Laboratory features	Serological features
AIH-PBC	Serum alkaline phosphatase (AP) level ≥2-fold upper limit of normal range (ULN)	AMA positive
AIH-PSC	AST/ALT>ULN γ-globulin and IgG >ULN AP or GGT>ULN	AMA negative
AIH-cholestatic syndrome	AST/ALT>ULN γ-globulin and IgG >ULN AP or GGT >ULN	AMA negative

References

↑ 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 (1999). “International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis”. J. Hepatol. 31 (5): 929–38. PMID 10580593.
↑ Czaja AJ (2008). “Performance parameters of the diagnostic scoring systems for autoimmune hepatitis”. Hepatology. 48 (5): 1540–8. doi:10.1002/hep.22513. PMID 18924244.
↑ Wiegard C, Schramm C, Lohse AW (2009). “Scoring systems for the diagnosis of autoimmune hepatitis: past, present, and future”. Semin. Liver Dis. 29 (3): 254–61. doi:10.1055/s-0029-1233532. PMID 19675998.
↑ Kuiper EM, Zondervan PE, van Buuren HR (2010). “Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome”. Clin. Gastroenterol. Hepatol. 8 (6): 530–4. doi:10.1016/j.cgh.2010.03.004. PMID 20304098.

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[pmid10580593-1] 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 (1999). “International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis”. J. Hepatol. 31 (5): 929–38. PMID 10580593.

[pmid18924244-2] Czaja AJ (2008). “Performance parameters of the diagnostic scoring systems for autoimmune hepatitis”. Hepatology. 48 (5): 1540–8. doi:10.1002/hep.22513. PMID 18924244.

[pmid19675998-3] Wiegard C, Schramm C, Lohse AW (2009). “Scoring systems for the diagnosis of autoimmune hepatitis: past, present, and future”. Semin. Liver Dis. 29 (3): 254–61. doi:10.1055/s-0029-1233532. PMID 19675998.

[pmid20304098-4] Kuiper EM, Zondervan PE, van Buuren HR (2010). “Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome”. Clin. Gastroenterol. Hepatol. 8 (6): 530–4. doi:10.1016/j.cgh.2010.03.004. PMID 20304098.

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