23例肺部受累的原发性小血管炎临床病理分析

Clinicopathologic analysis of 23 cases of primary vasculitis with lung involvement

目的 探讨两种累及肺部的原发性小血管炎 :Wegener s肉芽肿 (WG)和显微镜下多血管炎 (MPA)的临床特征 ,特别是呼吸道症状和影像学特征。方法 对 1998年～ 2 0 0 3年期间收治的WG和MPA患者进行回顾性分析。结果 WG发病年龄较MPA显著年轻。肺部受累的呼吸道症状分别是咳嗽 (10 0 % )、发热 (82 6 1% )、气促 (5 6 5 2 % )、咯血 (30 4 3% )和胸痛 (8 70 % )。发生呼吸衰竭者 39 13% ,肺部首发者 73 91%。WG胸部影像学以多发性肿块或结节阴影最常见 ,而MPA以双肺多发性片状影或间质纤维化最常见。常见的伴随症状是血尿、贫血、皮肤关节症状、鼻部症状和肝功能异常。WG常误诊为原发性或转移性肺癌 ,而MPA则常被误诊为肺部感染和间质性肺炎。结论 WG和MPA常累及肺部 ,其症状和胸部影像学为非特异性 ,因此在肺部肿瘤、感染和间质性肺炎的鉴别诊断中需注意除外原发性小血管炎WG和MPA。

Objective To explore the clinical and radiological features of Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) with lung involvement.Methods Retrospective analysis of WG and MPA cases admitted to Ruijin hospital in 1998～2003.Results WG was more frequent in young adults than MPA.Respiratory symptoms included cough (100%), fever (82.61%),dyspnea (56.52%),haemoptysis (30.43%) and chest pain (8.70%).Respiratory failure were occurred in 39.13% of all patients.Lung were first involved in 73.91% of all patients.Multiple mass and nodule was common lesions in WG,however,multiple opaque and interstitial fibrosis was common in MPA.The common accompanying manifestations included hematuria,anaemia,skin,joint and nasal symptom as well as abnormal liver function test.WG was usually misdiagnosed as primary lung cancer or metastasis while MPA was usually misdiagnosed as lung infection and interstitial pneumonia.Conclusions Lung were frequently involved in WG and MPA whithout characteristic clinical and radiological presentations.Primary vasculitis should be considered in the differentiation of lung tumor,infection and interstitial pneumonia.