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Gastroenterology, Hepatology, and Nutrition

G18 AUTOIMMUNE LIVER DISEASE/SYSTEMIC LUPUS ERYTHEMATOSUS OVERLAP SYNDROME IN CHILDREN
M. Samyn, D. Bogdanos, Y. Ma, B. Portmann, D. Vergani, G. Mieli-Vergani.Institute of Liver Studies, King’s College Hospital, London, UK

Background: An overlap syndrome between autoimmune liver disease (AILD) and systemic lupus erythematosus (SLE) has been occasionally described in adults, but never in children.

Patients: Between 1973 and 2004, eight (7 female) children who presented to our centre with AILD were also diagnosed with SLE. Median age at presentation was 8.75 (range 2.7–12.4) years. Duration of symptoms ranged between 0.5 and 12 months (median 1 month), the most common being jaundice (7), fever (5), lethargy (5), vomiting (4), joint (3), and abdominal pain (3). All children had high IgG (20.8–68.9 g/l, median 40.1 g/l). Five children had antinuclear antibody (ANA) and smooth muscle antibody (SMA), one ANA only and one SMA only, while one was ANA, SMA, and liver kidney microsomal1 (LKM1) antibody positive. Liver histology showed severe inflammation . . . [Full text of this article]