A case of eosinophilic granulomatosis with polyangiitis which bilateral sublingual painless swelling occurred first
A case of eosinophilic granulomatosis with polyangiitis which bilateral sublingual painless swelling occurred first
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以双侧舌下无痛性肿胀为先发的嗜酸性肉芽肿性多血管炎一例
DOI：
10.11277/stomatology.68.294
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
岡本 哲治
中科院分区：
文献类型：
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作者：
信本 忠義;神田 拓;林 靖也;谷 亮治;小川 郁子;岡本 哲治
嗜酸性肉芽肿病伴多血管炎（EGPA）属于抗中性粒细胞胞浆抗体相关性血管炎，是一种以毛细血管和小静脉等小血管病变为主的坏死性血管炎。它是一种罕见的系统性血管炎疾病。目前日本国内受影响的人数在2000人左右，估计每年新增患者100人。< br>这种疾病通常被称为Churg-Strauss综合征或过敏性肉芽肿性血管炎，但在2012年它被重新命名为EGPA。在头颈部区域，双侧对称的唾液腺肿胀已被证明。治疗主要以类固醇治疗为主，预后良好，但偶尔也有肠穿孔、心力衰竭等严重并发症的报道。< br>我们报告一例EGPA显示IgG 4相关疾病。在我们咨询风湿病和结缔组织病科后，类固醇治疗后，唾液腺肿胀等症状消失，但在首次治疗后4个月，观察到全身症状，如疲劳，面部水肿，上下肢皮肤发红，外周血IgE，IgG 4和嗜酸性粒细胞重新升高。因此，我们再次咨询该部门并诊断为EGPA。
Eosinophilic granulomatosis with polyangiitis (EGPA) is classified as anti-neutrophil cytoplasmic antibody associated vasculitis, which is a necrotizing vasculitis with mainly lesions in small blood vessels such as capillaries and venules. It is a rare systemic vasculitis disease. The number of people affected in Japan is currently around 2000, and it is estimated that there are 100 new patients each year.< br> The disease had been conventionally called Churg-Strauss syndrome or allergic granulomatous vasculitis, but in 2012 it was renamed EGPA. In the head and neck region, bilaterally symmetrical salivary gland swelling has been shown. Treatment is mainly based on steroid therapy and has a good prognosis, but occasionally there are reports of severe complications such as intestinal perforation and heart failure.< br> We report a case of EGPA showing IgG4-related disease. The symptoms such as salivary gland swelling disappeared with steroid therapy after we consulted the department of rheumatology and connective tissue disease, but four months after the initial treatment, systemic symptoms such as fatigue, facial edema, skin redness in the upper and lower limbs and re-elevation of peripheral blood IgE, IgG4 and eosinophil were observed. Accordingly, we consulted the department again and diagnosed EGPA.