Liver Transplantation for Pediatric AIH Type 2 at Age 7
Liver transplantation should be considered for a 7-year-old with AIH type 2 when presenting with acute liver failure with encephalopathy or when developing decompensated cirrhosis with complications of end-stage liver disease that cannot be salvaged with medical therapy. 1
Primary Indications for Transplant Evaluation
Acute Liver Failure
- Transplant is indicated when acute liver failure presents with encephalopathy, regardless of AIH subtype 1
- However, a trial of high-dose immunosuppression (prednisone 1-2 mg/kg/day up to 60 mg daily, with or without azathioprine) should be attempted first, as 90% of pediatric patients with liver failure from AIH can recover with medical therapy 2
- The likelihood of treatment success can be determined within 2 weeks: favorable indicators include resolution of at least one laboratory abnormality or improvement in hyperbilirubinemia 3
- If no improvement or worsening occurs after 2 weeks of corticosteroids, proceed urgently to transplant evaluation 3
Decompensated Cirrhosis
- Transplant is indicated when MELD score reaches ≥15 1
- Complications requiring transplant consideration include: 1
- Refractory ascites
- Variceal bleeding
- Hepatic encephalopathy
- Progressive synthetic dysfunction despite optimal medical therapy
Treatment Failure
- Transplant should be considered when there is inadequate response or intolerance to immunosuppressive therapy 1
- Treatment failure occurs in 5-15% of children with AIH, with AIH type 2 carrying a more severe prognosis than type 1 4, 3
- Failure to normalize transaminases despite escalated immunosuppression (high-dose prednisone 60 mg daily or prednisone 30 mg daily plus azathioprine 150 mg daily) indicates need for transplant evaluation 1
Special Considerations for AIH Type 2 in Children
Disease Severity
- AIH type 2 is more aggressive than type 1, with higher relapse rates and more frequent progression to cirrhosis 4
- More than 50% of children with AIH present with established cirrhosis at diagnosis 4
- Type 2 AIH (anti-LKM positive) is more frequently associated with fulminant liver failure than type 1 1
Post-Transplant Outcomes
- Five-year survival is excellent at 75-92%, with 10-year survival of 75% 1, 3
- However, disease recurrence occurs in 10-35% of pediatric patients post-transplant, with AIH type 2 showing an 80% recurrence rate compared to lower rates in type 1 4, 5
- Recurrence is more common in children than adults and may be less responsive to treatment 3, 5
Post-Transplant Management Requirements
- Children with AIH require more immunosuppression post-transplant than other pediatric recipients 1
- Maintenance steroids should NOT be discontinued post-transplant, as discontinuation accelerates disease recurrence 1, 5
- Tacrolimus is preferred over cyclosporine (71% recurrence rate with cyclosporine vs 18% with tacrolimus) 5
- Combination therapy with prednisone plus calcineurin inhibitor (preferably tacrolimus) is standard 1
Clinical Pitfalls to Avoid
Delayed Referral
- Do not wait until MELD score is very high before transplant evaluation—refer when MELD reaches 15 or when complications of portal hypertension develop 1
- Children with prolonged prothrombin time at presentation are at higher risk for eventual transplant need 5
Premature Transplant Decision
- Always attempt a trial of immunosuppression first, even in severe presentations, as 90% can recover with medical therapy 2
- Infection is the most frequent cause of morbidity and mortality during treatment of liver failure, not the liver disease itself 2
Inadequate Post-Transplant Immunosuppression
- Never discontinue steroids post-transplant in AIH patients, as this dramatically increases recurrence risk 1, 5
- Ensure families understand the need for lifelong, intensive immunosuppression before proceeding with transplant 1