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Characterization of Kikuchi-Fujimoto Disease in Children and Risk Factors Associated with Its Course - 08/09/23

Doi : 10.1016/j.jpeds.2023.113515 
Sujin Choi, MD 1, 2, Hyoung Soo Choi, MD 1, 2, Young Jin Ryu, MD 3, Ji Young Kim, MD 3, Jin Ho Paik, MD 4, 5, Soyeon Ahn, PhD 6, Hyunju Lee, MD 1, 2,
1 Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Republic of Korea 
2 Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea 
3 Department of Radiology, Seoul National University Bundang Hospital, Seongnam, Republic of Korea 
4 Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Republic of Korea 
5 Department of Pathology, Seoul National University College of Medicine, Seoul, Republic of Korea 
6 Department of Medical Research Collaborating Center, Seoul National University Bundang Hospital, Seongnam, Republic of Korea 

Reprint requests: Hyunju Lee, MD, PhD, Department of Pediatrics, Seoul National University Bundang Hospital and Seoul National University College of Medicine, Seoul, 03080, Korea.Department of PediatricsSeoul National University Bundang Hospital and Seoul National University College of MedicineSeoul03080Korea

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.

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Keywords : histiocytic necrotizing lymphadenitis, pediatric, recurrence, severe

Abbreviations : FANA, HLH, KFD, LN, NSAID, SLE


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Vol 260

Article 113515- septembre 2023 Retour au numéro
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