A rare presentation of systemic lupus erythematosus with lupus hepatitis

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. Prevalence of SLE in children and adolescents is 1-6 per 100,000 population. Liver dysfunction occurs in approximately in 50-60% of the patients with SLE.And the incidence of lupus hepatitis in diagnosed SLE patients is 9.3%. We are reporting a case of 11-year-old female child who presented with fever, skin rashes, oral ulcers, irritability and positive antinuclear antibody (ANA) with deranged liver function test (LFT) in the form of elevated transaminases, bilirubin level and altered coagulation profile. Hepatitis serology was negative, with low C3 levels, diagnosed as SLE with lupus hepatitis with lupus nephritis stage IV with psychosis. Clinical improvement along with improvement in terms of laboratory findings was seen on corticosteroids therapy. It is important to differentiate lupus hepatitis from autoimmune hepatitis (AIH) as AIH presents similar to lupus hepatitis, has poor prognosis. While lupus hepatitis being rare cause for mortality in patients with SLE has good response with early intervention with corticosteroids therapy.

Liver Involvement During Flare-ups in Pediatric SLE: Lupus Hepatitis vs. Other Causes

“…Systemic lupus erythematosus (SLE) is a complex autoimmune disease that involves many organs marked by circulating autoantibodies directed against selfantigens. It has a wide range of clinical manifestations and often mimics other diseases [1,2]. It can be affect any organ of the body, including the liver.…”

“…It is often reported with higher disease severity, morbidity and mortality than adult-onset disease. Childhood-onset SLE has an incidence rate of 0.3-0.9 per 100,000 children and a prevalence rate of 1-6 per 100,000 population [2,3]. SLE-associated hepatitis, or commonly known as lupus hepatitis, is a rare manifestation of SLE that involve liver dysfunction which is well documented, but considering rare that occurs in approximately 3-8% of SLE patients and is generally marked by mild elevated liver enzymes, but it can also occur in more severe degrees [1,4].…”