Symptom-based Kikuchi disease subtypes: Clinical scenarios across specialties in Taiwan with temporal trends analysis

Abstract

Background: We propose a subtyping system for Kikuchi disease based on chief complaints and fever status. Methods: A chart review of 388 patients diagnosed with Kikuchi disease. Results: The subtypes afebrile lymphadenopathy (aLAP), febrile lymphadenopathy (FebLAP), and febrile accounted for 68 %, 18 %, and 14 % of cases, respectively. aLAP patients were older (median 26 years), predominantly female, had fewer laboratory abnormalities, and a lower recurrence rate (5 %). In contrast, the febrile type included younger patients (median 17 years), predominantly male, with more laboratory abnormalities and a higher recurrence rate (20 %). FebLAP exhibited intermediate characteristics. Otolaryngology had the highest number of patients (272, 70 %), mainly with aLAP, typically diagnosed via outpatient needle biopsy, with a short follow-up duration. Infectious disease specialists (adult and pediatric) managed 67 patients (17 %), often encountering the febrile type, with patients frequently seen in the emergency room or hospitalized, diagnosed via surgical biopsy, and followed up more intensively and over longer periods. Approximately 9 % of patients were referred to rheumatology; these patients more frequently used disease-modifying antirheumatic drugs and steroids and were followed for an extended duration. From 2005 to 2022, the incidence of Kikuchi disease has doubled, driven by otolaryngologists’ aggressive use of ultrasound-guided core needle biopsy to diagnose more aLAP cases. Conclusions: Patients of different subtypes exhibit distinct characteristics, including demographic and laboratory data, recurrence rates, medical-seeking behaviors, diagnostic methods, treatments, and follow-up approaches, underscoring the clinical significance of this subtyping system. Changes in biopsy methods have led to the diagnosis of more aLAP cases.