以肺部表现首发的抗中性粒细胞胞浆抗体相关性小血管炎临床分析

Clinical analysis of antineutrophil cytoplasimc autoantibodies related vasculitis with pulmonary manifestations as the initial symptom

目的探讨以肺部表现首发的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎的临床特征。方法回顾分析10例以肺部症状首发的ANCA相关性小血管炎患者的临床资料。结果10例患者均有肺病变,肾脏损害5例,皮肤损害1例。所有患者ANCA阳性。双肺病变8例,单侧肺部病变2例。大部分患者可见血白细胞升高、贫血、血沉明显增快、C反应蛋白升高等。应用糖皮质激素及免疫抑制剂治疗,死亡3例,完全缓解7例。结论ANCA相关性小血管炎临床表现缺乏特异性,ANCA检查有助于该病的早期诊断。糖皮质激素及免疫抑制剂治疗能改善预后。

Objective To study the clinical features of antineutrophil cytoplasimc autoantibodies （ANCA） associated vasculitis with pulmonary manifestations as the initial manifestation. Methods Clinical data of 10 cases of ANCA-associated vasculitis manifested as pulmonary disorders were retrospectively analyzed. Results All the 10 cases had lung involvement, 5 cases had kidney disorders, 1 case had skin involvement. All of them were ANCA positive. 8 cases had shadows in both lungs, the other 2 cases only in half lung. Laboratory examination revealed anemia and elevated WBC, ESR and CRP. All patients were treated with corticosteroid and cyclophosphamide. 3 cases died, and the others had complete remission. Conclusion The clinical manifestation of ANCA associated vasculitis is not specific. ANCA test should be performed in order to make an early diagnosis. Administration of corticosteroid and immune suppressors can improve the prognosis.