Case Presentation: A 10-year-old Hispanic female with 7 years of episodic cervical lymphadenopathy and fever, presented with typical episode, new abdominal pain and emesis. Review of systems was positive for weight loss, night sweats, and diarrhea; she denied rash, cough, sore throat, and arthralgia. History was notable for many prior antibiotics, recently azithromycin and fluconazole for thrush, supplement shake intake, and travel to Mexico 7 years ago. Admission exam found thin, ill-appearing, but non-toxic child with normal vital signs, cervical lymphadenopathy, prominent parotids, few dental caries, no oral lesions, mild, diffuse abdominal tenderness without organomegaly, and no skin or joint findings. Labs were significant for WBC 3.4 K/uL, hemoglobin 9.7 g/dL, ESR >140 mm/hr, AST 620 U/L, ALT 614 U/L, total bilirubin 4.0 mg/dL, direct bilirubin 3.5 mg/dL, and lipase 82 U/L. Extensive infectious workup was negative including viral, fungal, and mycobacterial studies. ANA returned positive 1:1280 speckled, anti-dsDNA negative, and ENA panel pending. Anti-LKM and smooth muscle antibodies, cerulosplasmin, alpha-1 antitrypsin, acetaminophen level and urine toxicology screen were negative. Lymph node biopsy showed reactive hyperplasia. Liver biopsy suggested biliary pattern of injury, drug-induced versus obstruction. Magnetic resonance cholangiopancreatography unexpectedly found acute pancreatitis and repeat lipase was 9452 U/L! Intravenous methylprednisolone had been started 3 days prior, post-biopsies, with 4 criteria met for systemic lupus erythematosus (leukopenia, direct coombs, low complement, ANA). Finally, anti-Smith antibody returned positive confirming diagnosis of lupus, also positive anti-RNP, Ro/SSA, and La/SSB suggesting mixed connective tissue disease and Sjogren’s overlap.

Discussion: History of lymphadenopathy and fever, common pediatric complaints, guided this case with an initial broad differential diagnosis, including infection, malignancy and rheumatologic disease. The diagnostic course led to the correct diagnosis of lupus as the etiology of this patient’s complicated course and pancreatitis. Systemic lupus erythematosus (SLE) is a multisystem disease, often with gastrointestinal involvement, but rarely acute pancreatitis. The rate of pancreatitis with SLE is estimated 0.7%-4%. Pathogenesis may include vasculitis, micro-thrombi, anti-pancreatic antibodies, T-cell and complement-mediated inflammation. SLE associated pancreatitis has correlated with high activity of disease, and if coinciding with SLE symptoms mortality has been reported up to 40%. In several reports, as in ours, gastritis secondary to steroids was first assumed and delayed diagnosis. While steroids have been implicated in cases of pancreatitis in the general population, in SLE this association is not seen, and instead steroids demonstrate mortality benefit.

Conclusions: Providers should keep non-infectious etiologies, including SLE in mind when evaluating patients with recurrent lymphadenopathy and fever. We should also recognize risks associated with antibiotic prescribing, as the cause of liver injury was questioned in this case. Diagnosis of SLE must be made on clinical suspicion, aided by published clinical and immunologic classification criteria. Pancreatitis is a rare, dangerous complication of SLE not to miss. Its diagnosis and treatment with immunosuppression is critical, though should first exclude more common mechanical and toxic-metabolic etiologies.