Case Presentation: A 13-year-old previously healthy boy presented to the emergency department with 1 day of jaundice and scleral icterus as well as acute on chronic progressive abdominal pain. Review of systems was notable for unintentional 5 kg weight loss over the past year, fatigue, oral ulcers, Raynaud’s phenomenon, and gross hematuria. He had no fevers, arthralgias, or rashes. On exam, his vital signs were within normal limits with height and weight at the 75th percentile. He appeared sallow with scleral icterus, epigastric and RUQ tenderness with guarding, positive Murphy’s sign, and hepatomegaly. Initial laboratory workup was notable for normocytic anemia without signs of hemolysis (Hb 9.4 g/dL, Hct 26.4%, MCV 77.4 fL, reticulocyte count 2.65%), leukopenia with lymphopenia (WBC 3.41 K/uL, ALC 0.46 K/uL), elevated ESR 89 mm/hr, pancreatitis (lipase 4732 u/L), hepatitis (AST 1408 u/L, ALT 340 u/L), direct hyperbilirubinemia (total bilirubin 8.11 mg/dL, conjugated bilirubin 4.08 mg/dL), coagulopathy (PT 20.7 sec, PTT 63 sec, INR 1.9), acute kidney injury (BUN 22 mg/dL, Cr 0.90 mg/dL), and gross hematuria and nephrotic range proteinuria. Abdominal ultrasound with Doppler, abdominal CT, and MRI cholangiogram was notable for hepatosplenomegaly with splenic varices, markedly inflamed gallbladder without gallstones and borderline dilation of the common bile duct, severe pancreatitis, reactive adenopathy, and ascites. His constellation of symptoms was most concerning for severe liver dysfunction and risk of progression to acute liver failure, which in combination with multiorgan dysfunction raised concern for an autoimmune process. Further serologic workup revealed strongly positive ANA (1:2560), anti-Smith antibody (205 U), anti-double stranded DNA antibody (173 IU/mL), and hypocomplementemia (C3 36 mg/dL, C4 5 mg/dL) consistent with a new diagnosis of systemic lupus erythematosus (SLE). Kidney and liver biopsies later revealed class IV lupus nephritis and lupus hepatitis (LH), respectively. Additional workup for hepatitis including inherited metabolic disease (Wilson disease, alpha-1 antitrypsin deficiency), viruses (hepatitis A, B, C, E, CMV, EBV, HSV, HHV-6, enterovirus, adenovirus), drugs (acetaminophen, urine toxicology), and autoimmune hepatitis (AIH; anti-smooth muscle and anti-LKM antibodies) was negative.
Discussion: SLE is a chronic autoimmune inflammatory disease that presents in childhood (cSLE) in approximately 10% of cases. It can affect any organ system, most commonly involving the skin, muscle, blood, and kidney. Gastrointestinal (GI) symptoms occur at the time of diagnosis in 16% of cSLE, though GI features are not included in the diagnostic criteria for SLE. The prevalence of pancreatitis (0.7-4%), acalculous cholecystitis (0.5%), and LH (6%) in cSLE is rare. LH and AIH can both occur in SLE, and liver biopsy and serum antibodies can be used to differentiate the two diagnoses. There are important differences in prognosis and treatment, with AIH more often progressing to fulminant liver failure while LH is generally benign. While there are case reports describing severe liver dysfunction in AIH associated with cSLE, this is a unique case of severe liver dysfunction due to lupus hepatitis.
Conclusions: Severe liver dysfunction is a rare presentation of SLE but should be considered in patients with other manifestations of the disease and without another source of hepatitis. Liver biopsy and serum antibodies can further characterize the etiology of hepatitis, aiding in treatment.