Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. Due to its non-specific lymphadenopathy presentation, treatment is complicated by the frequency by which it is misdiagnosed-for example up to one-third of cases are misdiagnosed as malignant lymphoma, leading to expensive clinical testing and overtreatment of this typically self-limiting illness. KFD has a strong association with SLE, although its transience and rarity make it difficult to investigate. We present a case of KFD to illustrate the variance in presentation and typical outcome of KFD. We want to increase awareness and shed some light on some typical and atypical clinical presentations of KFD to reduce the incidence of misdiagnosis.
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http://dx.doi.org/10.1093/jscr/rjab442 | DOI Listing |
Immun Inflamm Dis
January 2025
Department of Pediatrics, NHO Okayama Medical Center, Okayama, Japan.
Aim: Kikuchi-Fujimoto disease (KFD) rarely affects pediatric patients and is characterized by prolonged fever and cervical lymphadenopathy. The diagnosis of KFD remains challenging and often requires an invasive biopsy. Low serum alkaline phosphatase levels have frequently been observed in patients with KFD; however, the clinical significance of low serum alkaline phosphatase levels remains unclear.
View Article and Find Full Text PDFOxf Med Case Reports
January 2025
Department of Pediatrics at Al-Baath Hospital, Al-Baath University, Al-Wehda, Homs, Syria.
Kikuchi-Fujimoto disease (KFD) is a rare condition first identified in Japan in 1972. It typically presents with high fever and lymph node swelling, and may be linked to autoimmune conditions or viral infections. A 6-year-old boy presented with cervical enlargement and recurrent high fever.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
March 2025
Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, New York Presbyterian Hospital, New York, NY, USA.
Purpose: We present a case of Kikuchi-Fujimoto Disease (KFD) associated with bilateral optic neuropathy progressing to vision loss.
Observations: A 17-year-old male was referred for bilateral optic nerve pallor. Eight years prior, he was diagnosed with KFD after workup for lymphadenopathy and treated with prednisolone acutely followed by long-term Plaquenil.
Cureus
December 2024
Internal Medicine, National Hospital of Sri Lanka, Colombo, LKA.
Kikuchi-Fujimoto disease is a rare systemic illness commonly affecting young females with a higher tendency to occur in the Asian population. Clinical presentation varies with most patients presenting with fever and cervical lymphadenopathy. The patient discussed in this case report presented to a tertiary care hospital in Sri Lanka with a fever for two weeks and palpable cervical lymphadenopathy.
View Article and Find Full Text PDFNat Genet
January 2025
Department of Statistical Genetics, Osaka University Graduate School of Medicine, Suita, Japan.
Aberrant immune responses to viral pathogens contribute to pathogenesis, but our understanding of pathological immune responses caused by viruses within the human virome, especially at a population scale, remains limited. We analyzed whole-genome sequencing datasets of 6,321 Japanese individuals, including patients with autoimmune diseases (psoriasis vulgaris, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), pulmonary alveolar proteinosis (PAP) or multiple sclerosis) and coronavirus disease 2019 (COVID-19), or healthy controls. We systematically quantified two constituents of the blood DNA virome, endogenous HHV-6 (eHHV-6) and anellovirus.
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