TABLE 3.

Empirical treatments and outcomes of the overlap syndromes of autoimmune hepatitis (AIH)

Overlap syndrome	Treatment	Outcomes
AIH-PBC (AP ≤2-fold ULN)	Prednisone or prednisolone (52): 30 mg daily × 1 week 20 mg daily × 1 week 15 mg daily × 2 weeks 10 mg daily thereafter	Normal or near-normal tests and liver tissue 81% (2) Treatment failure 14% (2)
	Combined with azathioprine (52): 50 mg daily from start, or 1 mg/kg/day to 2 mg/kg/day (European preference)
AIH-PBC (Paris criteria*)	Prednisone or prednisolone in combination with azathioprine as above combined with UDCA: 13 mg/kg/day to 15 mg/kg/day (19,30)	Serum AP, GGT and ALT levels improve (19,35) Limits hepatic fibrosis (35)
AIH-PSC (adults)	Prednisone or prednisolone 0.5 mg/kg/day tapered to 10 mg/day to 15 mg/day Combined with azathioprine 50 mg/day to 75 mg/day Combined with UDCA 13 mg/kg/day to 15 mg/kg/day (5,30,31,72)	Laboratory improvement variable in adults (2,18,27,38,72) Uncertain survival advantage in adults (20)
AIH-cholestatic syndrome	Prednisone or prednisolone in combination with azathioprine as above combined with UDCA: 13 mg/kg/day to 15 mg/kg/day (2,11,27)	Uncertain response (2,11,27) Disease progression 17% (2) Transplantation 33% (2)

Numbers in parentheses refer to references.

^*Paris criteria include interface hepatitis and either serum alanine aminotransferase (ALT) level ≥5-fold upper limit of normal range (ULN), immunoglobulin G level ≥2-fold ULN or smooth muscle antibodies and two of the following, serum alkaline phosphatase (AP) level ≥2-fold ULN or serum gamma glutamyl transferase (GGT) level ≥5-fold ULN, antimitochondrial antibodies and destructive cholangitis. PBC Primary biliary cirrhosis; PSC Primary sclerosing cholangitis; UDCA Ursodeoxycholic acid