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Kikuchi-Fujimoto disease in children: clinical features and disease course
Tai-Ju Wang1, Yao-Hsu Yang, Yu-Tsan Lin
1Department of Pediatrics, Cathay General Hospital, Taipei, Taiwan, ROC.
Insights
Kikuchi-Fujimoto disease (KFD) in children often presents with posterior cervical lymphadenitis and leukopenia. Pediatric KFD patients have a high risk of developing autoimmune syndromes, necessitating long-term monitoring.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Infectious Diseases
Background:
- Kikuchi-Fujimoto disease (KFD) is a rare condition causing cervical lymphadenitis, predominantly in Asian women, but also seen in children.
- Understanding KFD's pediatric presentation and long-term outcomes is crucial for effective management.
Purpose of the Study:
- To analyze the clinical manifestations and disease course of Kikuchi-Fujimoto disease in pediatric patients.
- To identify factors associated with a complicated disease course and the risk of developing autoimmune conditions.
Main Methods:
- Retrospective chart review and telephone interviews were conducted.
- Data were collected from 13 pediatric patients diagnosed with KFD between January 1988 and January 2003.
Main Results:
- Posterior cervical lymph node involvement occurred in 12 patients; leukopenia was observed in 9.
- All patients presented with C-reactive protein levels below 5 mg/dL.
- A high antinuclear antibody titer correlated with a more protracted and complicated KFD course.
- Five patients developed new symptoms suggestive of autoimmune processes, most commonly neurological symptoms, during follow-up.
Conclusions:
- Pediatric KFD carries a significant risk of evolving into an autoimmune syndrome.
- Close, long-term observation is mandatory for pediatric KFD patients to monitor for autoimmune complications.
Abstract:
Kikuchi-Fujimoto disease (KFD) is a rare cause of cervical lymphadenitis which mostly affects Asian women but is sometimes observed in the pediatric population. This study analyzed the clinical manifestations and disease course in children with KFD. Retrospective chart review and telephone interview were used to collect data for 13 children (8 boys and 5 girls) with a diagnosis of KFD from January 1988 to January 2003. Involvement of the posterior cervical lymph nodes was found in 12 patients, leukopenia in 9, and all patients had C-reactive protein less than 5 mg/dL. A high antinuclear antibody titer was associated with a more protracted and complicated course. Five of the 13 patients had new symptoms compatible with an autoimmune process during follow-up, with neurological symptoms the most common. In conclusion, the risk of evolution into an autoimmune syndrome in pediatric KFD patients is high, and careful long-term observation is mandatory.
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