Refractory autoimmune hepatitis in children: Considerations for assessment and management

Table 3 Scoring system by the European Society of Paediatric Gastroenterology, Hepatology and Nutrition for the diagnosis of juvenile autoimmune hepatitis

Criteria	Titre or level	Points
Positive autoantibodies1,2
Antinuclear antibodies (ANA) or anti-smooth muscle	≥ 1:20	+ 1
antibodies (SMA)	≥ 1:80	+ 2
Anti-liver kidney microsomal type 1 (anti-LKM1)	≥ 1:10	+ 1
	≥ 1:80	+ 2
Anti-liver cytosol type 1 (anti-LC1) antibody		+ 2
Anti-soluble liver antigen (anti-SLA)		+ 2
Anti-perinuclear neutrophil cytoplasmic antibody (pANCA)		+ 1
Immunoglobulin G (IgG)
	> ULN	+ 1
	> 1.2 × the ULN	+ 2
Histological features3
	Compatible with AIH	+ 1
	Typical of AIH	+ 2
Absence of viral hepatitis, Wilson disease, metabolic associated steatotic liver disease, drug exposure		+ 2
Presence of extrahepatic autoimmunity		+ 1
Family history of autoimmune disease		+ 1
Cholangiography
	Normal	+ 2
	Abnormal	- 2

¹Antibodies measured by indirect immunofluorescence on a composite rodent substrate (i.e., kidney, liver or stomach).

²Addition of points achieved for antinuclear antibody, smooth muscle antibody, anti-liver kidney microsomal antibody type 1 antibodies, liver cytosol antibody type 1, and anti-soluble liver antigen cannot exceed a maximum of 2 points.

³Typical histological features are those contained in the revised diagnostic criteria of the International Autoimmune Hepatitis Group, principally interface hepatitis.

AIH: Autoimmune hepatitis; ANA: Antinuclear antibody; SMA: Smooth muscle antibody; LKM-1: Liver kidney microsomal antibody type 1; LC-1: Liver cytosol antibody type 1; pANCA: Perinuclear antineutrophil cytoplasmic antibody; IgG: Immunoglobulin G; ULN: Upper limit of normal.