Clinical Manifestations, Therapeutic Response and Disease Outcome of Kimura's Disease in Taiwan

台灣木村氏症之臨床表現、治療反應以及疾病預後之分析

陳一銘(Yi-Ming Chen) ； 陳健行(Chien-Hsing Chen) ； 陳信華(Hsin-Hua Chen) ； 陳怡行(Yi-Hsing Chen) ； 陳得源(Der-Yuan Chen) ； 藍忠亮(Joung-Liang Lan)

《Formosan Journal of Rheumatology》 23卷1期 (2009/06) Pp. 33-39

https://doi.org/10.6313/FJR.2009.23(01).06

摘要

Objective: Kimura's disease (KD) is a rare chronic inflammatory disorder which is typically distributed among young oriental males. The aim of this study was to analyze the clinical characteristics, laboratory findings, therapeutic responses and disease outcome of 19 patients with KD in Taiwan.
Results: The mean age at onset was 26.2±17.2 years (range 3-58 years) and the majority were males (18/19, 95%). All patients had palpable masses around the head and neck region. Twelve (75%) of 16 patients who underwent laboratory examination had peripheral blood (PB) eosinophilia. Three patients had parotid gland involvement, three had nephrotic syndrome and one died of malignant lymphoma transformation. Among 17 patients with regular followed-up, seven (41%) of them experienced recurrence after surgery. The recurrence rate was higher among those with a younger age onset (mean age: 14.86±10.45 vs. 34.20±18.25, p＜0.01) and higher PB eosinophil count (2221.00±1431.13 vs. 769.28±594.89, p＜0.01) than those with an older age onset and lower PB eosinophil count. Conclusion: The involvement of salivary glands and kidneys suggest the systemic nature of KD, and a PB eosinophil count may be used as a predictor of recurrence in KD.

關鍵字

木村氏症 ； 臨床表現 ； 治療反應 ； 疾病預後 ； 嗜酸性白血球過多症

並列摘要

Objective: Kimura's disease (KD) is a rare chronic inflammatory disorder which is typically distributed among young oriental males. The aim of this study was to analyze the clinical characteristics, laboratory findings, therapeutic responses and disease outcome of 19 patients with KD in Taiwan.
Results: The mean age at onset was 26.2±17.2 years (range 3-58 years) and the majority were males (18/19, 95%). All patients had palpable masses around the head and neck region. Twelve (75%) of 16 patients who underwent laboratory examination had peripheral blood (PB) eosinophilia. Three patients had parotid gland involvement, three had nephrotic syndrome and one died of malignant lymphoma transformation. Among 17 patients with regular followed-up, seven (41%) of them experienced recurrence after surgery. The recurrence rate was higher among those with a younger age onset (mean age: 14.86±10.45 vs. 34.20±18.25, p＜0.01) and higher PB eosinophil count (2221.00±1431.13 vs. 769.28±594.89, p＜0.01) than those with an older age onset and lower PB eosinophil count. Conclusion: The involvement of salivary glands and kidneys suggest the systemic nature of KD, and a PB eosinophil count may be used as a predictor of recurrence in KD.

並列關鍵字

Kimura's disease ； clinical manifestations ； therapeutic response ； disease outcome ； eosinophilia