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{{Autoimmune hepatitis}}
{{Autoimmune hepatitis}}
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{{CMG}}; {{AE}} :{{MKK}}
 
 
==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]], [[SMA|SMAs]], [[Liver kidney microsomal type 1 antibody|LKM-1]], anti-liver cytosol 1 (anti-LC1) [[antibodies]], SLA/LP [[autoantibodies]] or [[ANCA|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
{| class="wikitable"
! colspan="2" |Revised Scoring System of the International Autoimmune Hepatitis Group
|-
|Laboratory Findings
|Points
|-
|
* AP:AST (or [[Alanine transaminase|ALT]]) ratio
** <1.5
** 1.5-3
** >3
|
<nowiki>+2</nowiki>
 
0
 
-2
|-
|
* Serum [[globulin]] or [[IgG]] level above ULN
** >2.0
** 1.5-2.0
** 1.5-1
** <1
|
+3
 
+2
 
+1
 
0
|-
|
* [[Antinuclear antibodies|ANA]], [[SMA]], or anti-LKM1
** >1:80
** 1:80
** 1:40
** <1:40
|
+3
 
+2
 
+1
 
0
|-
|
* AMA positive
|<nowiki>-4</nowiki>
|-
|
* [[Hepatitis]] markers
** Positive
** Negative
|
-3
 
+3
|-
|
* [[Hepatotoxic]] drug exposure
** Positive
** Negative
|<nowiki>-4</nowiki>
 
+1
|-
|
* Average [[alcohol]] intake (g/day)
** <25
** >60
|<nowiki>+2</nowiki>
 
-2
|-
|
* Histologic findings
** [[Interfaces|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]]
|<nowiki>+2</nowiki>
|-
|
* Other [[autoantibodies]]
|<nowiki>+2</nowiki>
|-
|
* [[HLA-DRB1|HLA DRB1*03]] or [[HLA-DRB1|DRB1*04]]
|<nowiki>+1</nowiki>
|-
|
* Response to [[Corticosteroid|corticosteroids]]
** Complete
** Relapse after drug withdrawal
|<nowiki>+2</nowiki>
 
+3
|-
| colspan="2" |
* 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
|-
| colspan="2" |AP, [[alkaline phosphatase]]; AST, [[Aspartate transaminase|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.<ref name="pmid10580593">{{cite journal |vauthors=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 |title=International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis |journal=J. Hepatol. |volume=31 |issue=5 |pages=929–38 |year=1999 |pmid=10580593 |doi= |url=}}</ref><ref name="pmid18924244">{{cite journal |vauthors=Czaja AJ |title=Performance parameters of the diagnostic scoring systems for autoimmune hepatitis |journal=Hepatology |volume=48 |issue=5 |pages=1540–8 |year=2008 |pmid=18924244 |doi=10.1002/hep.22513 |url=}}</ref><ref name="pmid19675998">{{cite journal |vauthors=Wiegard C, Schramm C, Lohse AW |title=Scoring systems for the diagnosis of autoimmune hepatitis: past, present, and future |journal=Semin. Liver Dis. |volume=29 |issue=3 |pages=254–61 |year=2009 |pmid=19675998 |doi=10.1055/s-0029-1233532 |url=}}</ref>
|}
'''Diagnostic features of the overlap syndromes of autoimmune hepatitis''' :<ref name="pmid20304098">{{cite journal |vauthors=Kuiper EM, Zondervan PE, van Buuren HR |title=Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome |journal=Clin. Gastroenterol. Hepatol. |volume=8 |issue=6 |pages=530–4 |year=2010 |pmid=20304098 |doi=10.1016/j.cgh.2010.03.004 |url=}}</ref>
{| class="wikitable"
! colspan="3" |Diagnostic features of the overlap syndromes of autoimmune hepatitis 
|-
| '''Type of overlap syndrome'''
|'''Laboratory features'''
|'''Serological features'''
|-
|AIH-[[Primary biliary cirrhosis|PBC]]
|'' S''erum [[alkaline phosphatase]] (AP) level ≥2-fold ''upper limit of normal range'' (ULN) '' ''
|[[Anti-mitochondrial antibody|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==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Needs content]]
 
[[Category:Hepatology]]
[[Category:Gastroenterology]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Gastroenterology]]
 
[[Category:Needs overview]]
{{WH}}
{{WS}}

Latest revision as of 17:28, 9 January 2018

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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:

Laboratory findings consistent with the diagnosis of autoimmune hepatitis include:

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

-3

+3

-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

+2
+2
+1
+2

+3

  • Aggregate score pretreatment
    • Definite autoimmune hepatitis - >15
    • Probable autoimmune hepatitis -10 -15
  • Aggregate score post-treatment
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

  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.
  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.
  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.
  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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