Kikuchi-Fujimoto disease, simultaneously diagnosed with systemic lupus erythematosus in an Arabic female: an agonizing combination

J Int Med Res. 2024 May;52(5):3000605241248884. doi: 10.1177/03000605241248884.

Abstract

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign condition affecting young Oriental-Asian females. It is characterized by fever and tender cervical lymphadenopathy with an unclear aetiology, and in most longitudinal reviews, KFD occurs before systemic lupus erythematosus (SLE). Herein, the case of a 28-year-old Kuwaiti female without any relevant past medical history, who was simultaneously diagnosed with KFD and SLE following an Ebstein-Barr virus infection, is reported. The patient was treated with oral prednisolone, hydroxychloroquine, cyclosporin, and belimumab and her response was clinically and biochemically favourable. Although KFD is prevalent in Asian populations, it may affect all races. Early diagnosis of KFD is difficult, particularly when simultaneously diagnosed with SLE, but crucial to preventing inappropriate therapy. Clinicians need to know about this rare disease, especially when patients present with fever and swollen lymph nodes, due to a risk of misdiagnosis with tuberculosis or lymphoma, as these are more often thought to be the cause of such symptoms.

Keywords: Kikuchi–Fujimoto disease; comorbidity; histiocytic necrotizing lymphadenitis; lymphadenopathy; systemic lupus erythematosus; viral infection.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Arabs
  • Female
  • Histiocytic Necrotizing Lymphadenitis* / diagnosis
  • Histiocytic Necrotizing Lymphadenitis* / drug therapy
  • Histiocytic Necrotizing Lymphadenitis* / pathology
  • Humans
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / drug therapy
  • Prednisolone / administration & dosage
  • Prednisolone / therapeutic use

Substances

  • Prednisolone