Macrophage activation syndrome in children with Kikuchi-Fujimoto disease
Authors' conclusion
The presence of KFD-MAS in children may be more common than previously expected, especially in those with skin rash. KFD-MAS may be associated with a higher recurrence rate. An extremely elevated serum LDH level and moderate to severe lymphopenia may be useful diagnostic markers for MAS in KFD.
Study context
- Population
- Children with histologically diagnosed Kikuchi-Fujimoto disease (KFD), Shenzhen Children's Hospital, January 2015 to November 2021histological diagnosis of KFD
- Participants
- 58 children
- Exposure
- KFD with macrophage activation syndrome (MAS) vs KFD without MAS
- KFD-MASn = 15
- Outcome
- Incidence and clinical features of MAS in KFD
Methodological detailssingle-centre, ROC curve analysis
- Single-centre
- ROC curve analysis
Main finding
As reported in the abstract
Incidence of MAS in KFD
KFD-MAS
25.9%
15
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Clinical features, KFD-MAS vs without MASKFD-MAS vs Without MAS | P = 0.01 |
| Clinical features, KFD-MAS vs without MASKFD-MAS vs Without MAS | P = 0.003 |
| Clinical features, KFD-MAS vs without MASKFD-MAS vs Without MAS | P = 0.04 |
| Laboratory findings, KFD-MAS vs without MASKFD-MAS vs Without MAS | P = 0.02 |
| Laboratory findings, KFD-MAS vs without MASKFD-MAS vs Without MAS | P = 0.002 |
| Laboratory findings, KFD-MAS vs without MASKFD-MAS vs Without MAS | P = 0.01 |
| Laboratory findings, KFD-MAS vs without MASKFD-MAS vs Without MAS | P < 0.0001 |
| Laboratory findings, KFD-MAS vs without MASKFD-MAS vs Without MAS | P < 0.0001 |
ROC cutoff values for KFD-MAS diagnosis
- Absolute lymphocyte count
- < 1235/μL
- Absolute platelet count
- < 171 × 106/μL
- Serum albumin
- < 35.6 g/L
- Serum lactate dehydrogenase (LDH)
- > 679 IU/mL
Original publication
Macrophage activation syndrome in children with Kikuchi-Fujimoto disease. Pediatr Rheumatol Online J. Published 25 January 2023. doi:10.
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