Nakagawa A, Yamaguchi T, Amano H, Takao T
Department of Respiratory Medicine, JR Tokyo General Hospital, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1995 May;33(5):543-7.
A 42-year-old housewife was admitted to our hospital because of an asthma attack, fever, severe eosinophilia, mononeuritis-multiplex, arthralgia, skin-eruptions, transient pulmonary infiltration, and other symptoms. Chung-Strauss syndrome was diagnosed. When there were signs and symptoms of vasculitis, chest radiography revealed that pulmonary infiltrates had decreased, but her chest CT showed ground glass opacities of both lung fields and fine granular shadows. Differential cell count of bronchoalveolar lavage fluid revealed a high level of eosinophils, and transbronchial lung biopsy specimens showed vasculitis and marked infiltration of eosinophils. When signs and symptoms of vasculitis were prominent, antineutrophil cytoplasmic antibodies (ANCA) in the serum were examined. PR3-ANCA (antibodies to serine-protease, nearly equal to c-ANCA), which is specific to Wegener's granulomatosis, was negative, but MPO-ANCA (antibodies to myeloperoxidase, nearly equal to p-ANCA) was positive and the level was very high (478 EU/ml). After corticosteroid therapy, her symptoms markedly improved, and MPO-ANCA became negative. MPO-ANCA appeared to reflect the disease activity in this case.
一名42岁的家庭主妇因哮喘发作、发热、严重嗜酸性粒细胞增多、多发性单神经炎、关节痛、皮疹、短暂性肺部浸润及其他症状入院。诊断为Chung-Strauss综合征。当出现血管炎的体征和症状时,胸部X线检查显示肺部浸润有所减轻,但胸部CT显示双肺野磨玻璃影及细颗粒状阴影。支气管肺泡灌洗液细胞分类计数显示嗜酸性粒细胞水平升高,经支气管肺活检标本显示血管炎及嗜酸性粒细胞明显浸润。当血管炎的体征和症状突出时,检测血清中的抗中性粒细胞胞浆抗体(ANCA)。对韦格纳肉芽肿具有特异性的PR3-ANCA(抗丝氨酸蛋白酶抗体,近似于c-ANCA)为阴性,但MPO-ANCA(抗髓过氧化物酶抗体,近似于p-ANCA)为阳性且水平非常高(478 EU/ml)。皮质类固醇治疗后,她的症状明显改善,MPO-ANCA转为阴性。在该病例中,MPO-ANCA似乎反映了疾病活动情况。