Abstract
Background: Generalized lymphadenopathy with fever, pulmonary involvement, and organomegaly in children can be caused by infections, malignancies, or autoimmune disorders, with infections being the most frequent. However, childhood-onset systemic lupus erythematosus (cSLE) can present with similar features, making diagnosis challenging in the absence of classical signs.
Case presentation: A previously healthy adolescent boy presented with a two-month history of intermittent high-grade fever, progressive bilateral neck swelling, and significant weight loss. Examination showed pallor, generalized lymphadenopathy, and hepatosplenomegaly. Laboratory investigations revealed anemia, positive Coombs test, elevated ESR, and ferritin. Infectious workup was negative. Imaging showed lung infiltrates and lymphadenopathy. Lymph node biopsy was reactive, without evidence of malignancy. Autoimmune panel came as positive for ANA and anti-dsDNA, with low complements, meeting ACR/EULAR (American College of Rheumatology/European League Against Rheumatism) criteria for SLE. Although hematuria was absent, nephrotic-range proteinuria was documented with preserved renal function. Renal biopsy confirmed class V lupus nephritis. He was treated with IV methylprednisolone, oral steroids, and Tacrolimus for induction therapy. At six months, the patient was clinically stable with no systemic symptoms and showed SLEDAI 4 with proteinuria improving.
Conclusion: This case highlights the diagnostic challenge of cSLE when presenting with features mimicking infection or malignancy. Systematic exclusion of differentials enabled timely diagnosis. Despite being asymptomatic, the patient had pulmonary nodules on imaging, and renal involvement was present without hematuria—reinforcing that cSLE can affect multiple organs early. Prompt recognition and immunosuppressive therapy led to clinical stability, emphasizing the need to consider autoimmune causes in atypical pediatric presentations.
Keywords
SLE, Lupus nephritis, Generalized lymphadenopathy, Hepatosplenomegaly, Lung nodule