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

Abstract

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.

Patients with treated autoimmune hepatitis and persistent suppression of plasmacytoid dendritic cells: A different point of view

“…Finally, a previous study has shown that Systemic Lupus Erythematosus (SLE) and AIH can reach 14% of similarity in their pathogenesis, 30 an overlap that has been described in pediatric patients. 32 It is speculated that DNA and RNA release by apoptotic cells in patients with SLE are potent stimuli to pDCs, via TLR-7 and -9 activation and high IFN-I production. Patients with SLE were shown to have increased peripheral IFN-I levels and activated pDCs in the spleen, 33 raising the question whether increased pDCs in peripheral blood samples of our patients could be caused by non-identified SLE, which should be investigated, although the association between pDCs, AIH and SLE remains unclear.…”

Diagnosis of tuberculosis with autoimmune hepatitis–systemic lupus erythematosus overlap syndrome: a case report

“…AIH–SLE overlap syndrome rarely progresses to severe outcomes such as cirrhosis requiring liver transplantation, hepatocellular carcinoma, and death seen in AIH patients [ 28 ]. Overlap syndrome also requires an individualized approach to treatment as each case is unique [ 29 ]. As per the management recommendations for AIH [ 24 ] (treatment initiated in patients with aminotransferases ten times the upper limit of normal OR aminotransferases five times the upper limit of normal with gamma globulin levels at least two times the upper limit of normal) and class V lupus nephritis, while considering the tuberculosis status as well as improving LFTs, we decided not to administer steroids/immunosuppression.…”

A rare presentation of systemic lupus erythematosus with lupus hepatitis

“…7 Clinical presentation of AIH is similar to lupus hepatitis but it has poor prognosis while, lupus hepatitis being rare cause for mortality in patients with SLE who has good response with corticosteroid therapy. 2,5 on medication associated with skin rashes initially started on the face and gradually progressed to involve axillary region and back. No significant past history, family history, developmentally appropriate for age, immunized according to national immunization schedule.…”