Proposed scoring criteria for the diagnosis of juvenile autoimmune liver disease

ANA and/or SMA*
Anti-LKM-1* or
Anti-LC-1
Anti-SLA
pANNA
IgG
Liver histology
Absence of viral hepatitis (A, B, E, EBV), NASH, Wilson disease, and drug exposure
Presence of extrahepatic autoimmunity
Family history of autoimmune disease
Cholangiography

Result :
AIH Score : 15. Definite AIH
ASC Score : 11. Definite ASC

Note

*Antibodies measured by indirect immunofluorescence on a composite rodent substrate (kidney, liver, stomach).
Addition of points achieved for ANA, SMA, anti-LKM-1, anti-LC-1, and anti-SLA autoantibodies cannot exceed a maximum of 2 points.

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