Autoimmune hepatitis: Appraisal of current treatment guidelines

Table 1 Serologic markers of autoimmune hepatitis

ANA	Variably expressed with ASMA in type 1 AIH
	Heterogenous antigen profile
	No single staining pattern is pathognomonic for diagnosis of AIH
	Most useful when found with ASMA (diagnostic accuracy 74%)[20]
ASMA	Marker of type 1 AIH along with ANA
	Reacts to several cytoskeletal elements, especially F-actin.
	ELISA against F-actin as the substrate can be used instead of indirect immunofluorescence but may miss the diagnosis in 15% to 20% of cases[20]
Anti-SLA/LP	Only disease specific antibody with specificity of 99% for AIH
	Present in only 15% patients with AIH in the United States
	Known to have a defined antigen, SEPSECS. ELISA is the preferred methodology of testing
	Closely associated with HLA DRB1*03 and Anti-Ro/SSA
	Have prognostic value as it is associated with severe disease, higher risk of relapse and need for lifelong treatment
Anti-LKM1	Serologic marker for type 2 AIH.
	CYP2D6 is the target antigen. Shares homology with hepatitis C virus antigen
	Present mainly in children, worldwide. Rare in adults in the United States (< 4%)
	Associated with HLA DRB*07
Atypical pANCA	Common in type 1 AIH, and absent in type 2 AIH
	Associated with PSC, UC

ANA: Antinuclear antibodies; ASMA: Anti-smooth muscle antibodies; AIH: Autoimmune hepatitis; ELISA: Enzyme linked immunosorbent assay; Anti-SLA/LP: Anti-soluble liver antigen/liver pancreas antibody; SEPSECSA: Sep (phosphoserine) tRNA: Sec (selenocysteine) tRNA synthase; Ro/SSA: Ribonucleoprotein/Sjögren’s syndrome A protein; Anti-LKM1: Antibodies to liver kidney microsome type 1; pANCA: Perinuclear antineutrophil cytoplasmic antibodies; PSC: Primary sclerosing cholangitis; UC: Ulcerative colitis.

Table 2 Simplified diagnostic criteria of the International Autoimmune Hepatitis Group

Parameter	Discriminator	Score
ANA or ASMA	≥ 1:40	+ 11
	≥ 1:80	+ 21
Anti-LKM	≥ 1:40	+ 21
Anti-SLA/LP	Any titer	+ 21
Total IgG	> ULN	+ 1
	> 1.1 × ULN	+ 2
Liver histology	Compatible with AIH	+ 1
	Typical of AIH	+ 2
Absence of viral hepatitis	No	0
	Yes	+ 2

¹Addition of all points for autoantibodies must be done, maximum 2 points allowed. Definite autoimmune hepatitis (AIH) ≥ 7; Probably ≥ 6. Typical histology for AIH: Each of the following should be present, interface hepatitis, plasma cell infiltrates, emperiopolesis, and hepatic rosette formation. Compatible liver histology: Chronic hepatitis with lymphocytic infiltrations without typical features as above. AIH: Autoimmune hepatitis; ANA: Antinuclear antibody; ASMA: Anti-smooth muscle antibody; Anti-LKM: Anti-liver kidney microsomal antibody; Anti-SLA/LP: Anti-soluble liver antigen/liver pancreas antibody[18].