CAJ | 학술논문

目的探讨韦格纳肉芽肿病的临床特点,以提高对本病的认识.方法回顾性分析1992年4月至2012年5月确诊的50例韦格纳肉芽肿病患者的临床资料,包括临床表现,实验室、影像学及病理检查等.结果男22例,女28例,发病年龄为13～74岁,平均43.8岁.确诊时间为14 d～180个月,平均16.7个月.临床以眼(38％)、耳(20％)、鼻(64％)、口咽(26％)等上呼吸道及肺部(58％)和肾(44％)受累为主.胞质型抗中性粒细胞胞质抗体(cANCA)阳性率96％,核周型抗中性粒细胞胞质抗体(pANCA)阳性率12％.病理表现为坏死性肉芽肿性炎症、炎细胞浸润的血管炎.治疗以糖皮质激素和免疫抑制剂为主.结论韦格纳肉芽肿病的临床表现复杂多样,缺乏特异性体征,靶器官受损有多系统或器官病变的症候群,尤以上、下呼吸道及肾脏受累最多见,病理特点为坏死性肉芽肿和血管炎.
목적 탐토위격납육아종병적림상특점,이제고대본병적인식.방법 회고성분석1992년4월지2012년5월학진적50례위격납육아종병환자적림상자료,포괄림상표현,실험실、영상학급병리검사등.결과 남22례,녀28례,발병년령위13～74세,평균43.8세.학진시간위14 d～180개월,평균16.7개월.림상이안(38％)、이(20％)、비(64％)、구인(26％)등상호흡도급폐부(58％)화신(44％)수루위주.포질형항중성립세포포질항체(cANCA)양성솔96％,핵주형항중성립세포포질항체(pANCA)양성솔12％.병리표현위배사성육아종성염증、염세포침윤적혈관염.치료이당피질격소화면역억제제위주.결론 위격납육아종병적림상표현복잡다양,결핍특이성체정,파기관수손유다계통혹기관병변적증후군,우이상、하호흡도급신장수루최다견,병리특점위배사성육아종화혈관염.
Objective Explore the clinical features of Wegener' s granulomatosis,in order to improve the understanding of the disease.Methods Retrospective analysis of 50 cases diagnosed in April 1992 to May 2012,the clinical data of patients with Wegener' s granulomatosis,including clinical manifestations,laboratory,radiological and pathological examination.Results 22 males,28 females,age of onset is 13 74 years old,with an average age of 43.8 years.Diagnosis time for 14 days 180 months,an average of 16.7 months.Clinical eyes (38％),ear (20％),nose (64％),oropharynx (26％),upper respiratory tract and lungs (58％) and renal (44％) is mainly involved.Cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA) positive rate of 96％,perinuclear anti-neutrophil cytoplasmic antibodies (pANCA)-positive rate of 12％.Pathological manifestations of necrotizing granulomatous inflammation,vasculitis,inflammatory cell infiltration.Treatment mainly glucocorticoids and immunosuppressants.Conclusions Complex and diverse clinical manifestations of Wegener's granulomatosis,lack of specific signs,target organ damage how the system or organ disease syndrome,especially over the most common lower respiratory tract and renal involvement,the pathological features of necrotizing granulomatous vasculitis.