This article is more than 5 years old. Information may no longer be current.
Canadian children with autoimmune hepatitis more likely to have type 1
Click Here to Manage Email Alerts
While uncommon, type 1 autoimmune hepatitis was diagnosed 5.5 times more frequently than type 2 in Canadian children, according to data published in Pediatrics.
“In our study we found that the annual incidence of autoimmune hepatitis [AIH] across Canada was approximately 2 per million children,” Carolina Jiménez-Rivera, MD, director of liver services gastroenterology, hepatology and clinical nutrition at Children’s Hospital of Eastern Ontario, told Infectious Disease in Children. “There was significant difference in the geographical distribution with high occurrence in the eastern provinces, raising the suspicion of genetic and/or environmental triggers.
“Up to 20% of children may have established cirrhosis at diagnosis, which speaks about the chronicity and insidious pattern of this disease.”
Patients with type 1 AIH typically have anti-smooth muscle and antinuclear autoantibodies; type 2 is characterized by anti-liver kidney microsomal and anti-liver cytosol type 1 autoantibodies, the researchers wrote.
Jiménez-Rivera and colleagues performed a retrospective review of patients aged younger than 18 years who were diagnosed with AIH between January 2000 and December 2009. They excluded autoimmune sclerosing cholangitis and AIH secondary to minocycline. One hundred fifty-nine patients (60.3% female; 13.2% type 2 AIH) were included in the study.
The annual incidence of AIH was 0.23 per 100,000 children, and the median age for patients with types 1 and 2 AIH were 12 years and 10 years, respectively, (P = .03). Jaundice was present in 54% of patients, and 49% of patients reported abdominal pain. In patients with AIH, serum albumin (33 g/L vs. 38 g/L; P = .03) and platelet count (187,000 vs 249,000; P < .001) were lower, while the international normalized ratio was higher in patients with cirrhosis (1.4 vs. 1.2; P < .001).
Initial treatment included corticosteroids (80%), azathioprine (32%), and/or cyclosporine (13%). Treatment response at 1 year was complete in 90% of patients and partial in 3% of patients. Three percent of the cohort had no response, and 3% relapsed. Nine patients underwent liver transplantation, and four patients died at an average follow-up of 4 years.
“Autoimmune hepatitis is a rare progressive inflammatory liver disease that can affect children and adults, and diagnosis is crucial to initiate therapy,” Jimenez said. “Physicians should think about this condition when children experience symptoms of fatigue, abdominal pain and jaundice, particularly in the presence of other personal (35% of cases) and/or family history (25%) of autoimmune diseases.
“Early diagnosis and treatment are crucial to halt disease progression preventing the need for liver transplantation and/or death.” – by Will Offit
Disclosure: The researchers report no relevant financial disclosures.
Click Here to Manage Email Alerts