Neurological manifestations and complications of Kikuchi-Fujimoto disease: A comprehensive systematic review

Abstract

Background: Kikuchi-Fujimoto disease (KFD) is a self-limiting inflammatory condition primarily affecting young and pediatric patients of Asian descent. This systematic review aims to consolidate current knowledge on the neurological manifestations associated with KFD.

Methods: The present review followed the PRISMA guidelines and was registered on PROSPERO (CRD42024579757). PubMed, Scopus and CINAHL Ultimate were searched to identify relevant studies. We included case reports and case series detailing neurological manifestations and complications of KFD of any age/gender.

Results: The initial search identified 456 articles out of which seventy-five case reports and series were included, encompassing a total of 81 cases. The median age of the patients was 23 years (IQR: 15-30). Patients most frequently presented with fever, headache and signs of meningeal irritation. Most patients diagnosed with KFD showed signs of neurological complications at the initial presentation. Meningitis (commonly aseptic meningitis) was the most frequently reported neurological complication followed by encephalitis, encephalopathy and neuro-ophthalmological complications. All patients had lymphadenopathy and showed the characteristic histopathological picture for KFD on biopsy. Steroids and immunosuppressive agents remained the treatment of choice. Complication and symptom specific treatment for neurological findings was provided wherever deemed necessary. Almost every study reported an improvement in neurological complications post treatment.

Conclusion: Neurological manifestations and complications associated with KFD are diverse and have a significant impact on patients. The complications associated with KFD need to be promptly recognized and evaluated by clinicians. Further investigation into the long-term effects and treatment strategies for KFD is warranted.

Keywords: Histiocytic Necrotizing Lymphadenitis; Immunology; Lymphadenitis; Neuroimmunology; Rheumatology; Systemic Lupus Erythematosus.