To explore the diagnostic features of histiocytic necrotic lymphadenitis (HNL) combined with systemic lupus erythematosus (SLE).

The clinical data of a patient diagnosed with HNL and SLE who was admitted to the Emergency Department of the First Affiliated Hospital of Zhengzhou University on December 29, 2019 were retrospectively collected and analyzed, and relevant literatures were reviewed.

The child was a 14-year-old female with fever and left neck pain for 2 weeks, and with lymphadenectasis of bilateral neck and axilla. The laboratory examination showed reduced neutrophil count, urine protein (+ + ), and erythrocyte sedimentation rate at 82.00 mm/h. Lymph node biopsy suggested histiocytic necrotizing lymphadenitis. Anti-nuclear antibody, anti-double-stranded DNA antibody and anti-Sm antibody were positive. The patient was diagnosed with HNL combined with SLE and lupus nephritis. After immunosuppressive treatment, the child′s condition improved.

HNL is susceptible to relapse combined with SLE. Missed diagnosis of SLE may delay the treatment of the disease. In the treatment of patients with fever and lymphadenectasis, attention should be paid to screening antibodies about SLE.