Refractory autoimmune hepatitis in children: Considerations for assessment and management

Table 5 Immunosuppressive therapies used in children for refractory autoimmune hepatitis

Medication		Route of administration	Dose in children	Trough level	Adjuvant therapy indicated
Mycophenolate mofetil	Second line	Oral	10 mg/kg/dose twice daily; maximum dose 20 mg/kg/dose twice daily	Not applicable	Yes- steroid
Tacrolimus	Second or third line	Oral	0.05 mg/kg/day- adjusted as per trough level	Initial serum trough levels 6-8 ng/mL, tapering to 3-5 ng/mL	Yes- steroid
Cyclosporine A	Third or fourth line	Oral	4 mg/kg twice daily- adjusted as per trough level	Initial trough levels at 200-250 ng/mL, tapering to trough levels < 120 ng/mL after full biochemical remission	Yes- steroid
Sirolimus	Third/fourth line	Oral	Initial dose 1-2 mg/m2 body surface area	Trough level of 4 ng/mL to 8 ng/mL	Yes- steroid
Rituximab	Third/fourth line	Intravenous infusion	375 mg/m2 2-4 doses every alternate week or fortnightly; further doses of rituximab might be needed 4-6 monthly	Not measurable. Surveillance of CD20+ B-cells is recommended; immunoglobulin supplementation may be necessary	Yes- steroid
Infliximab	Third/fourth line	Intravenous infusion	5 mg/kg infliximab is infusions at 0, after two weeks, and after six weeks of initial infusion, and thereafter every four to eight weeks depending on laboratory and clinical course	Data not available	Yes- steroid