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目的探讨抗中性粒细胞胞浆抗体(antineutrophilcytoplasmicantibodies,,ANCA)相关性肺血管炎的临床表现及早期诊断要点。方法采用回顾性方法对19例ANCA相关性肺血管炎患者的临床资料进行分析。结果本组19例均有肺损伤,肾脏损害13例,肝脏损害5例,皮肤损害5例,心脏损害3例;胸部CT示11例有双肺弥漫性病变。血沉为(89.20±19.82)mm/1h,C-反应蛋白为(55.33±34.12)mg/L。ANCA及特异性靶抗原[髓过氧化物酶(MPO)及蛋白酶3(PR3)]检查阳性。应用糖皮质激素及免疫抑制剂治疗,7例肺部病变完全吸收,血尿、蛋白尿减轻6例,肾功能改善3例;其他脏器功能情况好转或趋于稳定,1例肾衰竭患者需血液透析支持治疗。结论ANCA相关性肺血管炎临床表现复杂,除肺部病变外,常累及多器官、多系统,ANCA及相应的特异性靶抗原检查有助于该病的早期诊断。早期给予糖皮质激素及免疫抑制剂干预治疗,能延长患者的生存时间,改善预后。
Objective To investigate the clinical manifestations and early diagnosis points of anti-neutrophil cytoplasmic antibodies (ANCA) -related pulmonary vasculitis. Methods The clinical data of 19 patients with ANCA-associated pulmonary vasculitis were analyzed retrospectively. Results All the 19 cases had lung injury, 13 cases of kidney damage, 5 cases of liver damage, 5 cases of skin damage and 3 cases of heart damage. The chest CT showed 11 cases of diffuse pulmonary lesions. ESR was (89.20 ± 19.82) mm / 1h, C-reactive protein was (55.33 ± 34.12) mg / L. ANCA and specific target antigen [myeloperoxidase (MPO) and protease 3 (PR3)] positive. Application of glucocorticoid and immunosuppressive therapy, 7 cases of lung lesions completely absorbed, hematuria, proteinuria in 6 cases, 3 cases of renal function improvement; other organs function improved or stabilized, 1 case of renal failure patients need blood Dialysis support treatment. Conclusions The clinical manifestations of ANCA-associated pulmonary vasculitis are complicated. In addition to pulmonary lesions, multi-organ, multi-system, ANCA and corresponding specific target antigens are helpful to the early diagnosis of the disease. Early treatment with glucocorticoid and immunosuppressive agents can prolong the survival time of patients and improve the prognosis.