Autoimmune paediatric liver disease

Table 1 Clinical, laboratory, and histological features at presentation of autoimmune hepatitis type 1, autoimmune hepatitis type 2, and autoimmune sclerosing cholangitis[120]

	Type 1 AIH	Type 2 AIH	ASC
Median age in year	11	7	12
Females (%)	75	75	55
Mode of presentation (%)
Acute hepatitis	47	40	37
Acute liver failure	3	25	0
Insidious onset	38	25	37
Complication of chronic liver disease	12	10	26
Associated autoimmune diseases (%)	22	20	48
Inflammatory bowel disease (%)	20	12	44
Family history of autoimmune disease (%)	43	40	37
Abnormal cholangiogram (%)	0	0	100
ANA/SMA (%)	100	25	96
Anti LKM1 (%)	0	100	4
pANCA (%)	45	11	74
Anti SLA (%)1	58	58	41
Increased IgG level (%)	84	75	89
Partial IgA deficiency (%)	9	45	5
Low C4 level (%)	89	83	70
Increased frequency of HLA DR*0301	Yes	No2	No
Increased frequency of HLA DR*0701	No	Yes	No
Increased frequency of HLA DR*1301	No	No	Yes
Interface hepatitis (%)	66	72	35
Biliary features (%)	28	6	31
Cirrhosis (%)	69	38	15
Remission after immunosuppressive Treatment (%)	97	87	89

AIH: Autoimmune hepatitis; ASC: Autoimmune sclerosing cholangitis; ANA: Anti-nuclear antibodies; SMA: Anti-smooth muscle antibody; LKM1: Liver kidney microsomal type 1 antibody; pANCA: Perinuclear anti-neutrophil cytoplasmic antibody; SLA: Soluble liver antigen; C4: C4 component of complement; HLA: Human leukocyte antigen.

¹Measured by radioligand assay;

²Increased in HLA DR*0701 negative patients.