SHM Converge Scientific Abstract ePoster Gallery-Severe Hepatitis as Initial Presentation of Childhood-Onset Systemic Lupus Erythematosus

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A previously healthy 13-year-old boy presented with 1 day of jaundice and scleral icterus, plus acute worsening abdominal pain. His history included 1 year of weight loss, fatigue, oral ulcers, Raynaud’s phenomenon, and gross hematuria, with fevers, arthralgias, and rashes also noted. Exam showed right upper quadrant and epigastric tenderness, guarding, positive Murphy’s sign, and hepatomegaly.<br /><br />Labs demonstrated severe multi-organ involvement: anemia, leukopenia, elevated inflammatory markers, marked transaminitis (AST 1408, ALT 340), hyperbilirubinemia, prolonged PT/PTT/INR, and very elevated lipase consistent with pancreatitis. Urinalysis showed RBCs and protein. Imaging revealed hepatosplenomegaly, splenic varices, inflamed gallbladder without gallstones, borderline common bile duct dilation, severe pancreatitis, ascites, and reactive adenopathy.<br /><br />A broad workup for infectious, toxic, metabolic, and autoimmune hepatitis was negative, including evaluation for viral hepatitis, Wilson disease, alpha-1 antitrypsin deficiency, acetaminophen toxicity, and autoimmune hepatitis markers. Given the systemic features, serologies supported childhood-onset systemic lupus erythematosus (ANA 1:2560, anti-Smith positive, anti-dsDNA elevated, low complement levels). Biopsies confirmed class IV lupus nephritis and lupus hepatitis.<br /><br />The case highlights a rare presentation of cSLE with severe liver dysfunction, pancreatitis, and gallbladder inflammation. The authors emphasize that lupus hepatitis should be considered in children with hepatitis plus other lupus features when more common causes are excluded, and that liver biopsy and antibody testing can help distinguish lupus hepatitis from autoimmune hepatitis, which has different prognosis and treatment implications.

Asset Subtitle

Lauren Benedetto

Meta Tag

Author List Anirudh Bandu, Lauren Benedetto, Vivian Lee

Category Clinical Vignettes

Concept Juvenile-onset systemic lupus erythematosus

Concept Systemic Lupus Erythematosus

Concept Liver Involvement

Concept Lupus Hepatitis

Concept Renal involvement

Distinguished Non-Finalist

Presenter Organization Children's Hospital Los Angeles

Presenting Author Lauren Benedetto

Track Pediatric

Keywords

childhood-onset systemic lupus erythematosus

lupus hepatitis

pancreatitis

jaundice

hepatitis

lupus nephritis

gallbladder inflammation

anti-dsDNA

ANA positive

hyperbilirubinemia

Juvenile-onset systemic lupus erythematosus

Systemic Lupus Erythematosus

Liver Involvement

Lupus Hepatitis

Renal involvement