TABLE 1.

Comparison of histologic, clinical, and laboratory findings in pediatric autoimmune liver disease

	Histologic features	Clinical findings	Laboratory studies
AIH	Portal and lobular inflammation, typically with prominent plasma cells Piecemeal hepatocyte necrosis (interface and/or lobular hepatitis) May have duct inflammation but not destruction Progressive fibrosis	Female predominance Range from asymptomatic to fulminant liver failure Type 1: more common, usually adolescents; more common to have PSC overlap Type 2: younger than type 1	AIH type 1: ANA and/or SMA positive, elevated IgG AIH type 2: anti‐LKM1 and/or anti‐LC1 positive
PSC	Cholestatic pattern of injury Fibro‐obliterative duct lesions (fibrous replacement of bile duct) Concentric “onion‐skin” periductal fibrosis	Correlation with ERCP/MRCP findings Male predominance Associated with inflammatory bowel disease (IBD)	+ANA or may be pauci‐immune
ASC	May have both chronic hepatitis and cholestatic patterns of injury (features of both AIH and PSC above)	Younger at presentation than classic PSC; associated with IBD	May be pANCA positive + SMA or other + serologies