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目的探讨表现为支气管扩张症的肺血管炎临床表现与诊断,以提高认识,减少误诊。方法结合本院2010—2012年收治的3例表现为支气管扩张症的肺血管炎临床资料及国内外相关文献进行综合分析。结果患者一,65岁,女,反复咳嗽、咳痰、喘息及咯血10余年,加重伴发热1 d入院,肺部CT示双肺多发支气管扩张并感染,ANCA滴度1∶320、c-ANCA++,在抗感染基础上加用糖皮质激素治疗后,症状、体征明显好转,肺部CT示扩张的支气管部分恢复正常。患者二,56岁,男,因反复咳嗽、咳痰、喘息10余年,加重1周入院,肺部CT示双肺支气管扩张并感染,ANCA滴度1∶1 000、c-ANCA+,虽经积极抗感染及加用糖皮质激素,患者症状渐加重,于入院24 h临床死亡。患者三,68岁,男,因咳嗽约20 d入院,既往高血压、糖尿病史12年,右下肺支气管扩张症大咯血手术史10年,术后病理为肺结核。肺CT示右下肺支气管扩张,ANCA滴度1∶1 000、c-ANCA+,再次阅其术后病理片子,考虑为非干酪样肉芽肿、支扩,可除外肺结核,给予抗炎、糖皮质激素等治疗,好转后出院。结论以支气管扩张症为表象的肺血管炎,容易误诊,对可疑患者应尽早行ANCA检查,必要时行组织学检查以确诊。
Objective To investigate the clinical manifestations and diagnosis of pulmonary vasculitis manifested as bronchiectasis in order to enhance awareness and reduce misdiagnosis. Methods Combined with the clinical data of 3 cases of pulmonary vasculitis manifested as bronchiectasis in our hospital from 2010 to 2012 and the related literature at home and abroad for comprehensive analysis. Results A 65-year-old female with recurrent cough, expectoration, wheezing and hemoptysis for more than 10 years were admitted to hospital for 1 d after exacerbation. Pulmonary CT showed multiple bronchodilation and infection in both lungs. ANCA titers of 1: 320 and c-ANCA ++ , On the basis of anti-infective plus glucocorticoid treatment, the symptoms and signs improved significantly, lung CT showed expansion of the bronchial part returned to normal. Patient two 56 years old, male, due to repeated cough, sputum, wheezing more than 10 years, increased 1 week admission, lung CT showed bronchiectasis and lung infection, ANCA titers 1: 1000, c-ANCA +, although positive Anti-infection and add glucocorticoid, the patient’s symptoms gradually increased, 24 h admitted to hospital clinical death. Patient III, 68 years old, male, hospitalized for about 20 days due to coughing, previous history of hypertension and diabetes mellitus 12 years, history of major right hemispheric bronchiectasis with hemoptysis 10 years and postoperative pathology as pulmonary tuberculosis. Lung CT showed the right lower lung bronchiectasis, ANCA titers 1: 1000, c-ANCA +, read the postoperative pathology film, consider non-caseous granulomas, branch expansion, except tuberculosis, anti-inflammatory, glucocorticoid Such treatment, improved after discharge. Conclusions Bronchiectasis with bronchiectasis is easy to be misdiagnosed. ANCA should be performed as soon as possible on suspicious patients. If necessary, histological examination should be performed to confirm the diagnosis.