Immunosuppressant therapy causes permanent recovery from liver failure in most children with autoimmune hepatitis (AIH), reports Miriam Cuarterolo et al. in the February issue of Clinical Gastroenterology and Hepatology.
AIH is a progressive, inflammatory liver disease that predominantly affects girls before puberty. Without treatment, it progresses to cirrhosis and liver failure. Children with AIH and acute liver failure are a challenge to treat—there have been reports that they might benefit from immunosuppression, but there is not much evidence for this approach. Drug therapy of patients with AIH, especially with immnosuppressants, is controversial, because of the risk of septic complications.
Cuarterolo et al. analyzed data from 50 children with AIH and liver failure that had not been previously treated. The patients were given prednisone, alone or in combination with cyclosporine.
Forty-five children (90%) recovered liver function in a median time of 24 days—the 2 treatment regimens were equally effective in reducing liver inflammation. Only 2 of the 45 patients who responded to immunosuppression eventually required liver transplantation, because of complications from portal hypertension. However, 3 of these children given immunosuppressants died from infection—the only independent factor that was significantly associated with delayed recovery from liver failure. Three of the 5 children that did not respond to the immunosuppressive therapy received liver transplants.
Most studies of outcomes of children with AIH retrospectively analyzed patients with a wide range of clinical and biochemical features. These studies led to the conclusion that patients with advanced cirrhosis and liver failure were not likely to respond to drug therapies and instead should undergo liver transplantation.
The study by Cuarterolo et al. differs in that it includes a homogeneous, treatment-naive cohort of children with AIH (with signs of chronic liver disease, high serum levels of bilirubin, and portal hypertension). This allowed the authors to compare the effects of 2 different treatment regimens, and conclude that immunosuppressive therapy allows liver transplantation to be avoided or delayed.
Read the article online:
Cuarterolo ML, Ciocca ME, López SI, et al. Immunosuppressive therapy allows recovery from liver failure in children with autoimmune hepatitis. Clin Gastroenterol and Hepatol 2011;9:145–149.e1.