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目的探讨 Wegener 肉芽肿病耳部表现的临床特征,组织病理学和实验室检测特点,做到早诊断和早治疗。方法回顾性分析14例 Wegener 肉芽肿病耳部表现的临床资料。结果14例中包括慢性化脓性中耳炎10例,其中合并周围性面神经麻痹2例;分泌性中耳炎3例;感音神经性聋1例。14例中3例慢性化脓性中耳炎,1例分泌性中耳炎和1例周围性面神经麻痹是 Wegener肉芽肿病的首诊表现,开始表现为耳部单独受累,余9例开始即有多器官受累表现,全部病例均呈现多系统多器官受累。14例组织病理学诊断均符合 Wegener 肉芽肿病的特点。13例抗中性粒细胞胞质抗体阳性,14例有红细胞沉降率升高,13例 C 反应蛋白升高。所有病例经糖皮质激素和环磷酰胺治疗后病情均得到控制。11例随访时间3年以上,病情稳定无复发。结论 Wegener 肉芽肿病的耳部表现常缺乏特异性,早期诊断有赖于临床表现、组织病理学检查和抗中性粒细胞胞质抗体的及时检测。规范使用糖皮质激素和环磷酰胺可以有效地控制病情。
Objective To investigate the clinical features, histopathology and laboratory features of the ear in Wegener’s granulomatosis and to make early diagnosis and early treatment. Methods The clinical data of 14 patients with Wegener’s granulomatosis were retrospectively analyzed. Results Of the 14 cases, 10 cases were chronic suppurative otitis media, including 2 cases of peripheral facial paralysis; 3 cases of secretory otitis media; 1 case of sensorineural deafness. Three cases of chronic suppurative otitis media, one case of secretory otitis media and one case of peripheral facial paralysis were the first manifestation of Wegener’s granulomatosis in 14 cases, with the onset of ear involvement alone and multiple organ involvement in the remaining nine cases All cases showed multiple system multiple organ involvement. Histopathological diagnosis of 14 cases were in line with the characteristics of Wegener granulomatosis. 13 cases of anti-neutrophil cytoplasmic antibodies positive, 14 cases of erythrocyte sedimentation rate increased, 13 cases of C-reactive protein increased. All cases of glucocorticoid and cyclophosphamide after treatment were controlled. Eleven patients were followed up for more than 3 years without any recurrence. Conclusion The ear of Wegener’s granulomatosis often shows no specificity. The early diagnosis depends on the clinical manifestations, histopathological examination and timely detection of anti-neutrophil cytoplasmic antibodies. Standardized use of corticosteroids and cyclophosphamide can effectively control the disease.