显微镜下多血管炎肺损害的诊断探讨

Analysis of diagnosis of microscopic polyangiitis with lung involvement

目的探讨显微镜下多血管炎(MPA)肺损害的临床表现及诊断条件,旨在提高对该病的警惕性和认识水平。方法回顾性分析1998年1月至2015年1月收治的38例MPA患者的临床资料,包括临床症状、实验室检查、影像学改变、病理学检查等。结果肺损害发生率高达94.74%(36/38),其中26.32%(10/38)曾被误诊为特发性肺间质纤维化(IPF);63.16%(24/38)的患者有发热;71.05%(27/38)有咳嗽、咳痰;84.21%(32/38)出现持续性贫血;92.10%(35/38)合并血尿、蛋白尿;79.41%(27/34)血沉增快,72.73%(24/33)C-反应蛋白升高;97.37%(37/38)的患者抗中性粒细胞胞浆抗体(ANCA)阳性;57.89%(22/38)的患者影像学表现为肺间质纤维化。结论 MPA肺损害发生率高,临床表现复杂多样,无特异性,肺间质纤维化及肺炎样浸润影为常见影像学表现,极易误诊为IPF等疾病;ANCA对MPA的诊断有重要价值。

Objective To investigate the clinical manifestation and diagnosis of microscopic polyangiitis（ MPA） with lung involvement,aiming at improving the vigilance and the ability for recognization.Methods Clinical symptoms,laboratory examinations,imaging changes and histopathologic examinations of38 MPA cases were studied retrospectively. Results The lung involvement rate reached up to 94. 74%（ 36 /38）,of which 26. 32%（ 10 /38） was misdiagnosed as idiopathic pulmonary fibrosis（ IPF）. Common symptoms of MPA include fever（ 63. 16%,24 /38）,cough（ 71. 05%,27 /38）,continuous anemia（ 84. 21%,32 /38）,hematuria and proteinuria（ 92. 10%,35 /38）. Most of the MPA patients had elevated ESR and CRP. The detections of anti-neurtrophil cytoplasmic antibodies（ ANCA） on 97. 37%（ 37 /38） of the patients were positive. And 57. 89%（ 22 /38） of these cases were diagnosed as pulmonary interstitial fibrosis by X-ray or chest computed tomography（ CT）. Conclusions The rates of lung involvement of MPA was very high. The clinical manifestations of MPA were complex and varied. On account of pulmonary interstitial fibrosis and pulmonary infiltration were the main imaging alterations, MPA was easy to be misdiagnosed as IPF or pneumonia. ANCA shows significant value in diagnosis of MPA.