Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches

Abstract

Background Kimura disease (KD) is a rare, chronic, inflammatory disorder characterized by painless subcutaneous masses, lymphadenopathy, elevated eosinophil counts and increased serum immunoglobulin E (IgE) levels. Due to the heterogeneity and rarity, current diagnostic and treatment approaches for KD are often delayed and have limited efficacy. Methods The clinicopathological data of 51 KD patients between January 2014 and December 2023 were retrieved and stratified based on age and mass size, respectively. A case ofrecurrent KD who responded well to a low dose of mepolizumab was described. Results We found that pruritus occurred in 45.8% of patients, with higher rates in older patients (P=0.033). Edema was observed in 29.2%, with higher prevalence in younger patients (P=0.010). The mean maximum mass size was 3.1cm, varying by age group (P=0.005). The mean time to diagnosis was 36 months, increasing with mass size (P=0.024). Meanwhile, we reported that one of these 51 patients was successfully treated with mepolizumab at a dose of 100mg every 4 weeks. Conclusion We conclude that pruritus and edema are the two main clinical manifestation of KD, and the size of mass is related to the time of diagnosis. Biologic agents targeting the Th2 pathway show promise for KD treatment.