Background: Systemic lupus erythematosus (SLE) may present with heterogeneous clinical manifestations in pediatric patients. Although infections are a major cause of morbidity and mortality in SLE, severe bacterial infections rarely represent the presenting clinical event leading to diagnosis. Case description: We report the case of a 13-year-old boy diagnosed with SLE with Sjögren’s syndrome overlap who presented with pneumococcal sepsis. The patient was admitted with high-grade fever and facial swelling, and blood cultures grew Streptococcus pneumoniae. Although an initial clinical response to antibiotic therapy was observed, fever subsequently recurred, accompanied by persistent systemic symptoms and progressive laboratory abnormalities. Further investigations revealed hematologic abnormalities, serosal involvement, renal disease, and a characteristic autoantibody profile. The patient fulfilled the 2019 ACR/EULAR classification criteria for SLE after comprehensive autoimmune evaluation. The overlap with Sjögren’s syndrome was supported by the autoantibody profile and imaging findings involving the parotid glands. Following treatment with intravenous methylprednisolone pulses, oral prednisone, hydroxychloroquine, and mycophenolate mofetil, the patient showed rapid clinical improvement and sustained remission. Conclusions: This case highlights that severe invasive bacterial infection may occasionally be the clinical circumstance that leads to the diagnosis of pediatric systemic lupus erythematosus. Persistent systemic inflammation or evolving multisystem involvement despite appropriate antimicrobial therapy should prompt consideration of an underlying autoimmune disease, even in patients without a prior history of immune dysfunction.
Accomando et al. (2026) studied this question.