AUTOIMMUNE LIVER DISEASE/SYSTEMIC LUPUS ERYTHEMATOSUS OVERLAP SYNDROME IN CHILDREN

Abstract

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, 8 (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. Median duration of symptoms was 1 (range 0.5-12) month, the most common being jaundice (7), fever (5), lethargy (5), vomiting (4), joint (3) and abdominal pain (3). Five children had antinuclear antibody (ANA) and smooth muscle antibody (SMA), 1 ANA only and 1 SMA only, while 1 was ANA, SMA and liver kidney microsomal1 (LKM1) antibody positive. Liver histology showed severe inflammation in all 4 children biopsied at presentation, and moderate (2) and mild (2) inflammation in children biopsied after starting treatment. Fibrotic and cirrhotic changes were seen in 6 and 1 respectively. Treatment with Prednisolone was started at a median time of 1 day after presentation (range 1-4). Azathioprine was added in 4 patients. Mycophenolate Mofetil replaced Azathioprine in 2 and Cyclosporin was used in 1. SLE was diagnosed between 1 week and 6.9 years (median 7 mo) after AILD. 8 children had skin, 7 joint, 4 renal, 4 haematological and 3 pleural/pericardial involvement. ANA became positive in all, anti-double-stranded DNA and extractable nuclear antigens in 4 and 2 respectively. C4 was low in 5. 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 up is 3.7 (range 0.2- 8.7) years. Six children have normal liver function tests while 2 continue to have mildly raised AST. Two patients required further pulse steroids because of SLE relapse. Conclusion: AILD/SLE overlap syndrome does exist in children with AILD. SLE can be present at diagnosis of AILD or develop during follow up and must be timely and aggressively treated.