Table 2.

Difficult treatment decisions before starting conventional corticosteroid therapy

Problem	Response
Acute severe (fulminant) presentation	Prompt institution of conventional corticosteroid therapy with prednisone monotherapy[44,51-53]
	Azathioprine, 50 mg/d, can be added later if treatment is to be continued for ≥ 3 mo[55]
	Liver transplantation evaluation if laboratory indices worsen at any time during treatment, especially progressive hyperbilirubinemia, or no improvement after 2 wk[56]
Asymptomatic mild or mild disease	Institute conventional corticosteroid therapy with prednisone in combination with azathioprine[58,55]
	Consider empirical treatment with budesonide, 3 mg tid, in conjunction with azathioprine, 50 mg/d, if preexistent osteopenia, diabetes, hypertension, obesity, or emotional instability[25,26]
Autoantibody-negativity	Exclude viral, drug, toxic, metabolic causes and celiac disease[31,43]
	Apply codified scoring criteria of IAIHG for probable or definite diagnosis[31,46]
	Institute conventional corticosteroid therapy with prednisone in combination with azathioprine or a higher dose of prednisone alone[19,47-50]
Overlap syndromes	Conventional corticosteroid therapy alone or in combination with azathioprine if serum alkaline phosphatase level < 2 times ULN[59-62]
	Add ursodeoxycholic acid, 13-15 mg/kg per day, to corticosteroid regimen if serum alkaline phosphatase level ≥ 2 times ULN[60,63]
	Consider ursodeoxycholic acid alone, 13-15 mg/kg per day, if predominant features of PBC with minimal features of autoimmune hepatitis[64,65]

IAIHG: International Autoimmune Hepatitis Group; ULN: Upper limit of the normal.