台灣木村氏症之臨床表現、治療反應以及疾病預後之分析

Clinical manifestations, therapeutic response and disease outcome of Kimura’s disease in Taiwan

木村氏症（Kimura’s disease）是一種罕見的發炎性疾病，典型的表現常在年輕的東方男性病患，本研究主要目的在於分析台灣19位木村氏症病患的臨床特徵，實驗室檢查表現，治療的反應與疾病預後。本研究病人之平均發病年齡是26.2 ± 17.2歲（範圍3-58歲）而大多數病人為男性（18/19, 95%）。所有的病人在頭頸部都可觸摸到腫塊。曾經接受抽血檢查的十六位病人之中有十二位病人（75%）有周邊血液嗜酸性白血球過多症，三位病人出現耳下腺腫大，三位病人有腎病症候群而一位病人死於惡性淋巴瘤。在規則門診追蹤的十七人之中，七位（41%）在手術切除頭頸部腫塊後疾病復發。其中以發病年齡較輕者（平均年齡14.86 ± 10.45 vs. 34.20 ± 18.25, p<0.01）或周邊血液嗜酸性白血球較高者（2221.00 ± 1431.13 vs. 769.28 ± 594.89, p<0.01），疾病復發的機會較高。整體而言，唾液腺腫大以及腎臟侵犯表示木村氏症本身是一個系統性發炎的疾病，而周邊血液嗜酸性白血球過多症可用來預測疾病的復發。

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.