Kimura Disease: Retrospective Analysis of 53 Cases and Three Mepolizumab-Responsive Cases

Wenjiao Zhu,1,* Lei Zhang,2,* Jing Zhang,3,* Wei Wang,4 Ye Xiang,1 Dingxian He,5 Jing Shi,1 Yiran Liang,1 Yuanping Shi,6 Xianhui Ning,7 Meiling Jin,1 Ling Ye1 1Department of Allergy, Zhongshan Hospital, Fudan University, Shanghai, 200032, People’s Republic of China; 2Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, 200032, People’s Republic of China; 3Department of Pathology, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200011, People’s Republic of China; 4Department of Neurosurgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200011, People’s Republic of China; 5Department of Neurology, Huashan Hospital, Fudan University, Shanghai, 200040, People’s Republic of China; 6Department of Endocrinology and Metabolism, Peking University People’s Hospital, Beijing, 100044, People’s Republic of China; 7Department of Otolaryngology, Zhongshan Hospital, Fudan University, Shanghai, 200032, People’s Republic of China*These authors contributed equally to this workCorrespondence: Meiling Jin, Email [email protected] Ling Ye, Email [email protected]: Kimura disease (KD) is a rare, chronic lymphoproliferative disorder, which is believed to be driven by a Th2 immune response, though its exact pathogenesis remains unclear. Despite various treatment approaches, no standardized therapy has been established. This study investigated the clinical features of 53 KD patients and evaluated the therapeutic potential of mepolizumab in three cases.Methods: A retrospective age-stratified analysis was performed on 53 patients diagnosed with KD at two tertiary medical centers in China, covering the period from January 2014 to December 2024. We also provided an in-depth case series of three patients who received mepolizumab treatment.Results: This study included 53 KD patients, predominantly male (88.7%) with a mean age of 41 years. The median time from symptom onset to diagnosis was 24 months, and the median lesion diameter was 3.0 cm. Pruritus was present in 52.8% of patients, with frequency increasing with age (P=0.015). Coexisting conditions included IgG4-related disease (11.3%), asthma (9.4%), and thrombotic events (9.4%). Lesions were bilateral in 66.0% and primarily located in the head and neck region (83.0%). Average eosinophil count was 2.09 × 109/L, and mean serum IgE level was 1069 IU/mL. Surgical excision (62.3%) is the most common initial treatment approach, followed by corticosteroids (15.1%). The overall recurrence rate is 60.4%. In the case series, three patients treated with different doses of mepolizumab showed favorable responses, including lymph nodes shrink, symptom control and eosinophil reduction.Conclusion: This study provided a comprehensive summary and age-stratified analysis of the clinical characteristics of KD, providing valuable insights into its pathophysiology and potential overlapping mechanisms. Additionally, the study presents clinical cases of three patients treated with mepolizumab, highlighting its potential as a promising therapeutic option for the management of KD.Keywords: Kimura disease, eosinophilia, type 2 inflammation, mepolizumab