抗中性粒细胞胞浆抗体相关小血管炎的靶抗原及其临床病理特点

目的明确中国人抗中性粒细胞胞浆抗体（ANCA）相关小血管炎的检出率、主要的特异性靶抗原及临床病理特点。方法对近年来送检的820份不同患者的血清行IIF-ANCA检测，阳性血清则进一步经抗原特异性ELISA明确靶抗原，并探讨ANCA相关小血管炎患者的临床病理特点。应用的6个高度纯化的ANCA靶抗原包括蛋白酶3（PR3）、髓过氧化物酶（MPO）、杀菌／通透性增高蛋白（BPI）、组蛋白酶G（CG）、乳铁蛋白（LF）和人白细胞弹力蛋白酶（HLE）。结果43/820（5．2％）为IIF-ANCA阳性，其中36/820（4．4％）为原发性小血管炎。c-ANCA8例，其中7/8识别PR3，1例同时识别LF；p-ANCA28例，其中24例识别MPO，5例识别BPI，2例识别CG，1例识别LF，部分血清可同时识别二到三个不同的抗原。无1例血清识别HLE。ANCA相关小血管炎临床上多表现为多器官损害，肾、肺最易受累，肾脏病理呈活动性病变。ANCA相关小血管炎患者多有中重度贫血及明显的血沉快。结论ANCA相关小血管炎在我国并不少见；其主要的特异性靶抗原为MPO和PR3；临床呈多系统损害。

The prevalence of ANCA in Chinese patients with clinically suspeted small vessel vasculitis, their target antigens and clinicopathological characteristics

To investigate the prevalence of ANCA in Chinese patients with clinical suspected small vessel vasculitis,their major specific target antigens and to analyze the clinicopatbological characteristics of patients with ANCA related vasculitis. Methods 820 sera sent to our laboratory for ANCA test were screened by IIF-ANCA and the opitive sera were further tested by antigen specific ELISA using 6 highly purified known ANCA antigens as solid phase ligands. These antigens were proteinase 3(PR3 ), myeloperoxidase(MPO), bactericidal /permeability-increasing protein(BPI), cathepsin G (CG),lactoferrin and human leukocyte elastase (HLE). The clinicopathological characteristics of patients with ANCA related vasculitis were also studied. Results 43/820(5.2%) were IIF-ANCA positive, and 36/820(4.4%)were Primary small vessel vasculitis, eight of the 36 were c-ANCA and 7/8 recognized PR3;the other 28 were P-ANCA and 24/28recognized MPO,5 sera recognized BPI, 2 sera recognized CG and 2 recognized lactoferrin, none of the sera recognized HLE. Clinically, in most patients with ANCA related vasculitis and multi-system manifestations, kidney and lungs were the major organs to be involved. Majority of the Patients with ANCA related vasculitis had moderate or severe anemia and a remarkable elevated ESR. Conclusions ANCA related vasculitis was not rare in China and the major antigens were MPO and PR3. Clinically,over half Patients with ANCA related vasculitis had multi-system involvement. For clinically suspected patients, ANCA should be tested earlier in order to make a earlier diagnosis.