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两例变应性肉芽肿性血管炎(AGA);Churg-Strauss综合征

[Two cases of allergic granulomatosis and angiitis (AGA); Churg-Strauss syndrome].

作者信息

Ohkubo M, Igari S, Yasuda C, Suzuki S, Funabasi Y, Nisimaki T, Morito T, Kasukawa R, Wakasa H, Nagao S

机构信息

Second Department of Internal Medicine, Fukusima Medical College.

出版信息

Ryumachi. 1990 Oct;30(5):409-14, 417; discussion 414-6.

PMID:2084865
Abstract

AGA is an angiitis syndrome that has some characteristic features, for example preceding asthma and polyneuritis. And histological findings are granulomatous angiitis or extravascular granuloma. We report two typical cases of AGA. Case 1; 51-year-old woman had been suffering from asthmatic dyspnea for one year and developed in 1987 multiple neuritis in her extremities. Eosinophilia and high level of IgE were noted. Pathologic diagnosis of the biopsied right calf muscle specimen was granulomatous angiitis. Case 2; 40-year-old woman had been suffering from asthmatic dyspnea for two years. She complained of severe cough and myalgia in 1986 and her chest X-ray showed homogeneous shadows in right upper and left lower fields. And her blood showed eosinophilia and high level of IgE. The histology of the biopsied subcutaneous nodules of hands showed extravascular granuloma. These two cases had specific features of AGA. About symptoms of angiitis, case 1 showed multiple neuritis and case 2 had subcutaneous nodules of hands. About laboratory data, case 1 showed WBC count of 9400/mm3 with 85% eosinophils and high level of IgE at 1400 IU/ml, case 2 had WBC count of 13200/mm3 with 22% eosinophils and IgE at 846 IU/ml. The vary of eosinophil count and IgE level were related to the degree and course of illness. These symptoms and laboratory data, except neuritis, improved by an administration of prednisolone. In early stage of AGA, prednisolone is effective, so the criteria of AGA and usage of corticosteroids must be considered.

摘要

变应性肉芽肿性血管炎(AGA)是一种具有某些特征性表现的血管炎综合征,例如前驱性哮喘和多发性神经炎。组织学表现为肉芽肿性血管炎或血管外肉芽肿。我们报告两例典型的AGA病例。病例1:一名51岁女性,哮喘性呼吸困难已持续一年,并于1987年出现四肢多发性神经炎。发现有嗜酸性粒细胞增多和高水平的免疫球蛋白E(IgE)。对活检的右小腿肌肉标本进行病理诊断为肉芽肿性血管炎。病例2:一名40岁女性,哮喘性呼吸困难已持续两年。1986年她主诉严重咳嗽和肌痛,胸部X线显示右上肺野和左下肺野有均匀阴影。她的血液检查显示嗜酸性粒细胞增多和高水平的IgE。对手部活检的皮下结节进行组织学检查显示血管外肉芽肿。这两例病例具有AGA的特异性表现。关于血管炎的症状,病例1表现为多发性神经炎,病例2有手部皮下结节。关于实验室数据,病例1白细胞计数为9400/mm³,嗜酸性粒细胞占85%,IgE水平高,为1400 IU/ml,病例2白细胞计数为13200/mm³,嗜酸性粒细胞占22%,IgE为846 IU/ml。嗜酸性粒细胞计数和IgE水平的变化与疾病的程度和病程有关。除神经炎外,这些症状和实验室数据经泼尼松龙治疗后有所改善。在AGA的早期阶段,泼尼松龙是有效的,因此必须考虑AGA的诊断标准和皮质类固醇的使用。

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