Case Report
Copyright ©2007 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. May 21, 2007; 13(19): 2764-2767
Published online May 21, 2007. doi: 10.3748/wjg.v13.i19.2764
An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood
Yusuf Usta, Figen Gurakan, Zuhal Akcoren, Seza Ozen
Yusuf Usta, Figen Gurakan, Hacettepe University, Faculty of Medicine, Department of Pediatrics; Section of Gastroenterology, Hepatology and Nutrition, Sihhiye 06100, Ankara, Turkey
Zuhal Akcoren, Hacettepe University, Faculty of Medicine, Department of Pediatrics, Pediatric Pathology Unit, Sihhiye 06100, Ankara, Turkey
Seza Ozen, Hacettepe University, Faculty of Medicine, Department of Pediatrics, Nephrology and Rheumatology Unit, Sihhiye 06100, Ankara, Turkey
Author contributions: All authors contributed equally to the work.
Correspondence to: Yusuf Usta, MD, Hacettepe University, Faculty of Medicine, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, Ankara 06100, Turkey. dryusta@yahoo.com
Telephone: +90-312-3051993 Fax: +90-312-3054157
Received: February 19, 2007
Revised: February 25, 2007
Accepted: March 21, 2007
Published online: May 21, 2007
Abstract

We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.

Keywords: Autoimmune hepatitis; Systemic lupus erythematosus; Overlap syndrome and treatment