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 in all four children biopsied at presentation, and moderate (2) and mild (2) inflammation in children biopsied after starting treatment. Fibrotic and cirrhotic changes were seen in six and one, respectively. Treatment with prednisolone was started at a median time of 1 day after presentation (range 1–4). Azathioprine was added in four patients. Mycophenolate mofetil replaced azathioprine in two and cyclosporin was used in one. SLE was diagnosed between 1 week and 6.9 years (median 7 month) after AILD. Eight children had skin, seven joint, four renal, four haematological, and three pleural/pericardial involvement. ANA became positive in all, anti-double stranded DNA and extractable nuclear antigens in four and two, respectively. C4 was low in five. Soluble liver antigen (SLA) antibodies were positive in 5/7, ribosomal P antibodies in 4/5 tested. SLE was treated with high dose pulse steroids (5), cyclophosphamide (4), hydroxychloroquine (3), methotrexate (2), and plasmapheresis (1). Median follow …