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Case Report ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Pediatr. | doi: 10.3389/fped.2019.00310

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review

 Wan Tz Lai1,  Wan-Hua Cho2, 3, Hock-Liew Eng4, Ming-Hui Kuo1 and  Fu-Chen Huang1, 3*
  • 1Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Taiwan
  • 2Department of Ophthalmology, Kaohsiung Chang Gung Memorial Hospital, Taiwan
  • 3College of Medicine, Chang Gung University, Taiwan
  • 4Department of Anatomic Pathology, Kaohsiung Chang Gung Memorial Hospital, Taiwan

Background: The diagnosis of overlap syndrome involving systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) is not easily established because of its similar clinical presentations and biochemical features to those of lupus hepatitis. The term overlap syndrome is usually used in the context of overlap of autoimmune hepatitis with PSC (primary sclerosing cholangitis) or PBC (primary biliary cholangitis). Few cases of AIH complicated by SLE have been reported in the literature, and the condition is even rarer in childhood.
Case presentation: Here we report the case of a 16-year-old girl with SLE who initially presented with autoimmune (cholestatic) hepatitis. According to American Association for the Study of Liver Diseases practice guidelines, the diagnosis was made based on aggregated scores including female (+2); ALP:AST (or ALT) ratio < 1.5(+2); elevated serum IgG level(+3); ANA > 1:80 (+3); negative hepatitis viral markers and drug history (+3, +1); average alcohol intake < 25 g/day (+2); and histological interface hepatitis features (+3). She then developed a malar rash, ANA positivity, anti-double-stranded DNA (anti-dsDNA) antibodies, and a low complement level. She met 4 of 17 Systemic Lupus International Collaborating Clinics classification criteria (1) for SLE. Our patient responded very well to corticosteroid at an initial dose of methylprednisolone 40 mg Q12H for 4 days tapering to 1 mg/kg/day according to liver function test results and bilirubin level. No relapse occurred during the 3-year follow-up course.
Conclusions: Overlapping of SLE and AIH should be suspected when children with SLE have impaired liver function or AIH patients present with a malar or other skin rash. Liver biopsy plays an important role in establishing the differential diagnosis of SLE with liver impairment or overlap with AIH. The prompt diagnosis and adequate further treatment plans can improve disease outcomes.

Keywords: Overlap syndrome, systemic lupus erythematosus (SLE), Autoimmune hepatitis (AIH), Jaundice, childhood

Received: 13 Nov 2018; Accepted: 09 Jul 2019.

Edited by:

Pascal Lapierre, University of Montreal Hospital Centre (CRCHUM), Canada

Reviewed by:

Isabel G. Costa, Hospital and University Center of Coimbra, Portugal
Tudor L. Pop, Iuliu Hațieganu University of Medicine and Pharmacy, Romania
Victor M. Navas-López, Hospital Materno-Infantil, Spain  

Copyright: © 2019 Lai, Cho, Eng, Kuo and Huang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD. Fu-Chen Huang, Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, 83301, Taiwan,