抗中性粒细胞抗体相关性血管炎31例临床分析

Clinical characteristics of 31 patients with ANCA-associated vasculitis.

目的分析抗中性粒细胞抗体（ANCA）相关性血管炎（AAV）伴肾脏损害患者临床特征。方法收集2002年9月至2007年9月四川大学华西医院收治的31例临床诊断为AAV患者。间接免疫荧光及免疫酶联吸附试验（ELISA）均为ANCA阳性。逐项分析ANCA抗原谱，肾脏和肾外临床表现。结果本组AAV患者男16例，女15例。年龄18—84岁，平均（54．19±20．00）岁。显微镜下多血管炎（MPA）27例，韦格纳肉芽肿（WG）4例。肾脏症状首发16例，肾外症状首发15例，以呼吸系统症状首发者8例，8例伴咯血。27例MPA患者就诊时血肌酐平均（460．42±354．55）μmol／L。全组患者P—ANCA阳性24例，C-ANCA阳性7例，抗原识别抗MPO阳性25例。抗PR3阳性6例，抗BPI阳性3例。结论AAV临床表现多样，肺、肾是最常见的受累器官。本病晚期治疗方法十分有限．预后甚差．重视ANCA筛查．旱期诊断是改善预后的关键。

Objective To investigate the clinical characteristics of 31 patients suffering from ANCA associated vaseulitis （AAV）. Methods The clinical data of 31 patients with ANCA positive profiles diagnosed as AAV were analyzed ,including ANCA spectrum,renal and other organs＇ clinical features. Results There were 16 males and 15 females,average admission age （54. 19 ±20. 00） years（ 18 to 84 years）. Totally 27 MPA and 4 WG were diagnosed;onset symptoms of renal involvement were in 15 cases and others in 16 cases;18 patients had respiratory system involvements including 8 cases with pulmonary hemorrhage. In admission 27 MPA patients with average SCr （460.42 ± 354. 55 ） μmol/L,and WG group with （659. 62± 535.1 ） μmol/L. ANCA spectrum showed 24 P-ANCA cases and 7 C-ANCA cases, while ELISA method showed 25 anti-MPO cases and 6 anti-PR3 cases. Conclusion AAV has many kinds of manifestations and progresses in many variable ways. Kidney and respiratory system are most vulnerable in AAV. The treatment is very limited in advanced stage of AAV. The key to improving the outcome of AAV is early detection of ANCA and early diagnosis.