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一例嗜酸性肉芽肿性多血管炎患者气道黏膜出现多处白色苔藓样病变。

A case of eosinophilic granulomatosis with polyangiitis showing multiple white lichen lesions on the airway mucosa.

作者信息

Kimura Yosuke, Ito Ryo, Hayashi Yoshiki, Kazawa Toshihiro, Endo Yoshiro, Iwashima Akira, Ohshima Yasuyoshi, Watanabe Satoshi, Koya Toshiyuki, Kikuchi Toshiaki

机构信息

Department of Respiratory Medicine and Infectious Disease, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-Dori, Chuo-ku, Niigata, 951-8510, Japan.

Department of Respiratory Medicine, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, 4132 Urasa, Minamiuonuma, Niigata, 949-7302, Japan.

出版信息

Respir Med Case Rep. 2021 Jun 29;33:101451. doi: 10.1016/j.rmcr.2021.101451. eCollection 2021.

Abstract

A 70-year-old man, treated for asthma for 2 years and chronic sinusitis for several months, presented with fever, numbness in the lower limbs, heaviness in the head, gross hematuria, and black stools. He also had eosinophilia, elevated serum IgG4 levels, high levels of myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA), and pulmonary infiltrative shadows. Bronchoscopy revealed multiple white flattened lesions (white moss) on the airway mucosa, suggesting mycobacterial infection or malignancy. A biopsy from tracheal mucosa revealed airway inflammation with marked eosinophil infiltration. The patient was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) and treated with steroids, and all findings improved. However, a year and a half after the initiation of treatment, eosinophils and IgE gradually increased; subjective symptoms, such as asthma symptoms and numbness in the lower limbs, worsened; and ANCA, which had been negative, turned positive. Therefore, we suspected disease relapse and anti-IL-5 antibody (mepolizumab) treatment was initiated. Thereafter, ANCA turned negative again, eosinophils and IgE normalized, and subjective symptoms decreased. The presence of airway mucosal lesions in EGPA is relatively rare, and we report this case as a valuable case owing to the interesting bronchoscopic findings that are worth comprehending as a respiratory physician.

摘要

一名70岁男性,患哮喘2年,慢性鼻窦炎数月,出现发热、下肢麻木、头部沉重、肉眼血尿和黑便。他还伴有嗜酸性粒细胞增多、血清IgG4水平升高、髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)水平升高以及肺部浸润阴影。支气管镜检查显示气道黏膜有多个白色扁平病变(白色苔藓),提示分枝杆菌感染或恶性肿瘤。气管黏膜活检显示气道炎症伴明显嗜酸性粒细胞浸润。该患者被诊断为嗜酸性肉芽肿性多血管炎(EGPA),并接受了类固醇治疗,所有症状均有改善。然而,治疗开始一年半后,嗜酸性粒细胞和IgE逐渐升高;哮喘症状和下肢麻木等主观症状加重;之前为阴性的ANCA转为阳性。因此,我们怀疑疾病复发,并开始使用抗IL-5抗体(美泊利单抗)治疗。此后,ANCA再次转为阴性,嗜酸性粒细胞和IgE恢复正常,主观症状减轻。EGPA中气道黏膜病变相对少见,我们将此病例作为一个有价值的病例报告,因为其有趣的支气管镜检查结果值得呼吸内科医生了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4050/8349094/30f87866e308/gr1.jpg

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