Auto immune hepatitis

Table 1 Studies of incidence and prevalence of autoimmune hepatitis

Ref.	Year	Cases	Incidence/100000	Prevalence/100000
Toda et al[10]	1997	496	0.8	-
Whalley et al[125]	2007	200	3.0	-
Werner et al[9]	2008	473	0.85	10.7
Grønbæk et al[7]	2014	1721	1.68	23.9
Gerven et al[8]	2014	1313	1.1	18.3
Ngu et al[39]	2010	138	2.0	24.5
Delgado et al[126]	2013	100	0.67	11.0
Primo et al[127]	2004	13	1.37	11.61
Hurlburt et al[11]	2002	77	-	42.9

Table 2 Presentation and symptoms in auto immune hepatitis

Acute hepatitis
Chronic hepatitis
Hepatomegaly
Splenomegaly
Spider naevi
Palmar erythema
Non specific symptoms:
Tiredness
Fever
Loss of appetite
Upper abdominal pain
Arthralgia
Extrahepatic autoimmune disease (most common mentioned):
Thyroiditis	10%-23%
Primary biliary cirrhosis	10%-20%
Diabetes	7%-9%
Primary sclerosing cholangitis	2%-8%
Rheumatoid arthritis	2%-5%
Celiac disease	1%-2%

Table 3 Simplified diagnostic criteria for auto immune hepatitis[75]

Variable	Cutoff	Points
ANA or ASMA	≥ 1:40	1
ANA or ASMA	≥ 1:80
or LKM-1	≥ 1:40	2
or SLA	Positive
IgG	> Upper normal limit	1
	> 1.10 times upper normal limit	2
Liver histology (evidence of hepatitis is a necessary condition)	Compatible with AIH	1
	Typical AIH	2
Absence of viral hepatitis	Yes	2
		≥ 6: probable AIH
		≥ 7: definite AIH

ANA: Antinuclear antibodies; ASMA: Anti-smooth-muscle antibodies; LKM-1: Anti Liver kidney microsomal; IgG: Immunoglobulin G.

Table 4 Indication for treatment of auto immune hepatitis (adapted from Manns et al[4])

Absolute	Relative
Serum AST ≥ 10 fold ULN	Symptoms (fatigue, arthralgia, jaundice)
Serum AST ≥ 5 fold ULN and IgG level ≥ twice normal	Serum AST and/or IgG less than absolute criteria
Bridging necrosis or multiacinar necrosis on histological examination	Interface hepatitis

AST: Aspartate transaminase; ULN: Upper limit normal; IgG: Immunoglobulin G.