Characterization of Kikuchi-Fujimoto Disease in Children and Risk Factors Associated with Its Course

J Pediatr. 2023 Sep:260:113515. doi: 10.1016/j.jpeds.2023.113515. Epub 2023 May 25.

Abstract

Objective: To outline the characteristics of Kikuchi-Fujimoto disease (KFD) in children and analyze factors associated with severe and recurring courses.

Methods: Electronic medical records of children histopathologically diagnosed with KFD at Seoul National University Bundang Hospital from March 2015 to April 2021 were retrospectively reviewed.

Results: A total of 114 cases (62 males) were identified. The mean patient age was 12.0 ± 3.5 years. Most patients came to medical attention with cervical lymph node enlargement (97.4%) and fever (85%); 62% had a high-grade fever (≥39°C). Prolonged fever (≥14 days) was seen in 44.3% and was associated with a high-grade fever (P = .004). Splenomegaly, oral ulcer, or rash was present in 10.5%, 9.6%, and 15.8%, respectively. Laboratory findings showed leukopenia, anemia, and thrombocytopenia in 74.1%, 49%, and 24%, respectively. Sixty percent of cases had a self-limited course. Antibiotics were initially prescribed in 20%. A corticosteroid was prescribed in 40% of patients and was associated with oral ulcer (P = .045) and anemia (P = .025). Twelve patients (10.5%) had a recurrence with a median interval of 19 months. No risk factor for recurrence was identified in multivariable analysis. Clinical characteristics of KFD were similar between our current and previous studies. However, antibiotics use decreased (P < .001); nonsteroidal anti-inflammatory drugs use increased (P < .001), and, although statistically not significant, corticosteroid treatment also increased.

Conclusions: Over a span of 18 years, the clinical characteristics of KFD did not change. Patients presenting with high-grade fever, oral ulcer, or anemia may benefit from corticosteroid intervention. All patients should be monitored for recurrence.

Keywords: histiocytic necrotizing lymphadenitis; pediatric; recurrence; severe.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Anti-Bacterial Agents / therapeutic use
  • Child
  • Fever / complications
  • Histiocytic Necrotizing Lymphadenitis* / complications
  • Histiocytic Necrotizing Lymphadenitis* / diagnosis
  • Humans
  • Leukopenia* / drug therapy
  • Male
  • Oral Ulcer* / complications
  • Oral Ulcer* / drug therapy
  • Retrospective Studies

Substances

  • Adrenal Cortex Hormones
  • Anti-Bacterial Agents