ArticlePediatric rheumatology online journal2023
Macrophage activation syndrome in children with Kikuchi-Fujimoto disease.
Article in Pediatric rheumatology online journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 10 citations in OpenAlex.
- Unveiling the mystery of prolonged fever with lymphadenopathy and skin rashes- Kikuchi-Fujimoto disease with discoid lupus erythematosus: A case report.JAAD case reports · 2026Article
- Diagnostic Challenges and Scientific Management of Suspected Recurrent Kikuchi-Fujimoto Disease: A Case Report.The American journal of case reports · 2026Article
- Kikuchi-Fujimoto disease mimicking hemophagocytic lymphohistiocytosis in a young white male: a case report.Journal of medical case reports · 2026Article
- From suspected lymphoma to self-limiting lymphadenitis: Kikuchi-Fujimoto disease in focus.Journal of hematopathology · 2026Article
- Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series.Frontiers in immunology · 2026Article
- A Kikuchi-Fujimoto syndrome in the pocket of a non-Hodgkin lymphoma: case report with a mini-review of the literature.Annals of medicine and surgery (2012) · 2025Article
- Eco-friendly Synthesis of Iron Oxide Nanoparticles Using Parietaria alsinifolia Extracts and Evaluation of Biological Applications.Applied biochemistry and biotechnology · 2025Article
- Recurrence of Histiocytic Necrotizing Lymphadenitis in Children: A 10-year Multicenter Retrospective Study.Journal of inflammation research · 2025Article
- Kikuchi-Fujimoto disease: 6 years old boy rare case in Syria.Oxford medical case reports · 2025Article
- Late progression of pediatric Kikuchi-Fujimoto disease to systemic lupus erythematosus: a case report and review of literature.Frontiers in immunology · 2025Review
- Clinical features and recurrence predictors of histiocytic necrotizing lymphadenitis in Chinese children.Pediatric rheumatology online journal · 2024Article
- Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.Internal and emergency medicine · 2023Article
- Elevated serum IFN-γand IFN-γ/IL-6 ratio in Kikuchi-Fujimoto disease.Pediatric rheumatology online journal · 2023Article
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
backgroundKikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS.
methodsPatients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children's Hospital. Clinical data were collected from inpatient or outpatient medical records. Data collected included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. Data were analyzed using GraphPad Prism 8.0 statistical software (GraphPad Software Inc., La Jolla, CA, USA). A receiver operating characteristic (ROC) curve analysis was further performed to access the potential predictors for the KFD-MAS diagnosis.
resultsOf 58 patients with a histological diagnosis of KFD, 15 (25.9%) patients had MAS. Compared to patients without MAS, patients with KFD-MAS presented with a higher proportion of skin rash (26.7%, p = 0.01), glucocorticoid treatment (80%, p = 0.003), and disease recurrence (33.3%, p = 0.04). KFD-MAS patients had lower absolute peripheral white blood cell (WBC, p = 0.02), platelet (p = 0.002), serum albumin levels (p = 0.01), and lymphocyte count (p < 0.0001), and higher lactate dehydrogenase (LDH) levels (p < 0.0001). ROC curve analysis showed that the cutoff values of absolute lymphocyte count, an absolute platelet count, serum albumin level, and serum LDH level for KFD-MAS diagnosis were < 1235/μL, < 171 × 10
conclusionsThe 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.
trial registrationNot applicable; this was a retrospective study.
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