Notes from NASPGHAN’s postgraduate course:
Refractory Autoimmune Hepatitis (AIH): — Vicky Ng
Dr. Ng’s talk started with an overview of AIH and referred to AASLD guidelines: Diagnosis and Management of Autoimmune Hepatitis – AASLD
Recommendations included the following:
- Cholangiography for all new cases of AIH
- Starting azathioprine after seeing some improvement in transaminases with steroids
- Monitoring for HCC
- Monitor bone density/bone protection strategies discussed
- Long term Rx needed in majority, though small number may be able to come off therapy if doing well for 2 years and normal liver biopsy
- This is applicable in 15-20% of patients
- Reasons for refractory disease: non response, drug intolerance, non-compliance, overlap syndrome, comorbidities
- If treatment failure, options could include increasing steroids and azathioprine. If concerns for decompensation, refer for liver transplant evaluation.
- NO standard Rx for refractory, but consider MMF (mycophenolate mofetil), cyclosporin (CYA), or tacrolimus (FK)
- MMF most promising agent for refractory disease. Small studies of MMF in adults/pediatrics indicates response in about 2/3rds of patients; best for those intolerant to azathioprine & helpful in dropping steroid dosing. In pediatrics, a starting dose of 20 mg/kg/day is typical and increasing up to 40 mg/kg/day. Pediatric study: 18/26 (69%) with response and 14/18 with normal AST w/in 2 months.
- Tacrolimus –small study showed about ~90% response. Dose was 0.1 mg/kg/day & target trough was 3 ng/mL
- Briefly discussed budesonide. More data in pediatrics needed.
Postgraduate Course Syllabus (posted with permission): PG Syllabus
Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician. Application of the information in a particular situation remains the professional responsibility of the practitioner.