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Pediatric Kikuchi-Fujimoto disease: A clinicopathologic study and the therapeutic effects of hydroxychloroquine
Yung-Chih Lin1, Hsiu-Hui Huang2, Bao-Ren Nong3
1Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.
Insights
Kikuchi-Fujimoto disease (KFD) in children often presents with febrile cervical lymphadenopathy. Hydroxychloroquine showed favorable effects and safety as a treatment option for KFD.
Area of Science:
- Pediatric Infectious Diseases
- Rheumatology
- Dermatology
Background:
- Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting condition that primarily affects lymph nodes.
- Accurate diagnosis and effective treatment strategies for pediatric KFD are crucial.
Purpose of the Study:
- To delineate the clinical characteristics of Kikuchi-Fujimoto disease in pediatric patients.
- To evaluate the efficacy and safety of hydroxychloroquine as a monotherapy for KFD in children.
Main Methods:
- Retrospective review of medical records for pediatric KFD cases diagnosed between January 1992 and September 2016.
- Analysis of clinical presentations, laboratory findings, histopathological features, and treatment outcomes.
Main Results:
- Forty pediatric patients with histopathologically confirmed KFD were analyzed (mean age 13.9 years).
- Common symptoms included cervical lymphadenopathy (95%), fever, cough, and leukopenia (56.5%) with monocytosis (63.6%).
- Hydroxychloroquine monotherapy in five patients resulted in no recurrences or significant adverse effects.
Conclusions:
- Febrile cervical lymphadenopathy with leukopenia and monocytosis should raise suspicion for KFD in children.
- Specific laboratory and histopathological findings can predict prolonged fever.
- Hydroxychloroquine presents a safe and effective alternative to corticosteroids for KFD treatment in children.
Background:
To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) in children, and place an emphasis on the therapeutic effects of hydroxychloroquine as monotherapy.
Methods:
We retrospectively reviewed the medical records of all children diagnosed with KFD during the period January 1992 to September 2016 at a tertiary medical center in Taiwan.
Results:
40 patients were histopathologically confirmed as KFD, and the mean age of the patients was 13.9 ± 3.1 years. The male to female ratio was 1:1. The lymph node involvements were often cervical (95%) with features of unilateral predisposition (75%), polyadenopathy (84.4%) and tenderness (56.3%). Fever, cough, rhinorrhea, and tonsillitis were other common presentations. Laboratory findings included leukopenia (56.5%), monocytosis (63.6%), with positive results of EB-VCA IgG (88.9%), EB-VCA IgM (22.2%), EBEA IgG (22.2%) and EBNA IgG (88.9%). The univariate analyses of prolonged fever with lymphopenia, monocytosis, thrombocytopenia and necrotizing type in histopathology were disclosed as statistically significant (P < 0.05). Corticosteroids and hydroxychloroquine were administered in 15.6% of patients respectively, along with symptomatic treatments for the rest. Recurrence occurred in 13.0% of patients without corticosteroids or hydroxychloroquine treatment. There were neither recurrences nor relevant major adverse effects in all the five KFD cases treated with hydroxychloroquine.
Conclusion:
KFD should be suspected in children with febrile cervical lymphadenopathy, especially when concomitant with leukopenia and monocytosis. Lymphopenia, monocytosis, thrombocytopenia and necrotizing type in histopathology are reliable predictors for prolonged fever. Hydroxychloroquine may be an alternative choice to corticosteroids for its favorable effects and safety.
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