Autoimmune hepatitis in childhood: A 20-year experience

Abstract

between the ages of 10 and 20 and 45 and 70 years and has To determine the clinical, biochemical, and histologia relatively benign course at least in adults, 3 whereas LKMcal features, and outcome of childhood autoimmune 1 positive patients have a more severe disease with progreshepatitis (AIH), we reviewed the medical records of 52 sion to cirrhosis despite immunosuppressive treatment. gest that ANA/SMA positive AIH has two peaks of incidence median age: 10.5, range 2.3-14.9 years). At presentation, four children were positive for ANA alone (titer range: 1:80-1:320), 10 for SMA alone (titer range: 1:40-1:2560) and 14 for both ANA (titer range: 1:20-1:5120) and SMA (titer range: 1:40-1:1280). In two paAbbreviations: AIH, autoimmune hepatitis; ANA/SMA, nuclear and/or smooth muscle

Autoimmune liver disease in Asian children

“…Three fourths of patients are girls, some 20 % have associated autoimmune disorders-including thyroiditis, vitiligo, type 1 diabetes, inflammatory bowel disease, IgA nephropathy-and about 40 % have a family history of autoimmune disease [2]. In children and adolescents, AIH has a more aggressive course than in middleage and elderly patients and often presents acutely, though its mode of presentation is very variable, and the disease should be suspected and excluded in all children with symptoms and signs of liver disease not ascribable to more common pathologies.…”

“…The epidemiology of childhood AIH is unknown, but AIH type 1 (AIH-1) [anti-nuclear antibody (ANA) and/or anti-smooth muscle antibody (SMA) positive] accounts for 2/3 of the cases and presents often around puberty [2], whereas AIH-2 [positive for anti-liver kidney microsomal antibody type 1 (anti-LKM1) and/or anti liver cytosol type 1 antibody (anti-LC1)] tends to present at a younger age and also during infancy. IgG is usually raised at presentation in both types, though 15 % of children with AIH-1 and 25 % of those with AIH-2 have normal levels.…”

“…IgG is usually raised at presentation in both types, though 15 % of children with AIH-1 and 25 % of those with AIH-2 have normal levels. IgA deficiency is common in AIH-2 [2]. Severity of disease is similar in the two types, but anti-LKM1-positive children usually have higher levels of bilirubin and transaminases at presentation than those who are ANA/SMA-positive and present significantly more frequently with fulminant hepatic failure [2].…”

“…IgA deficiency is common in AIH-2 [2]. Severity of disease is similar in the two types, but anti-LKM1-positive children usually have higher levels of bilirubin and transaminases at presentation than those who are ANA/SMA-positive and present significantly more frequently with fulminant hepatic failure [2]. Excluding children with the fulminant presentation, a severely impaired hepatic synthetic function (prolonged prothrombin time and hypoalbuminaemia) is usually more common in AIH-1 than in AIH-2.…”

“…Most series of juvenile autoimmune liver disease published to date comprise mainly Caucasoid patients [2][3][4][5][6][7][8][9].…”