CAJ | 학술논문

目的：探讨成年人原发性抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎（AASV）的特点并分析其预后，以提高对本病的认识。方法选取原发性 AASV患者51例，对其临床表现、辅助检查、病理结果、治疗及预后等资料进行分析。结果51例中，肺部受累39例、肾脏受累40例；实验室检查髓过氧化物酶（MPO）阳性45例、蛋白酶3（PR3）阳性6例；12例行肾脏病理检查，表现为毛细血管襻坏死和新月体形成。51例患者中49例给予糖皮质激素治疗（其中40例接受糖皮质激素联合免疫抑制剂治疗），1例仅予免疫抑制剂治疗，1例未及接受激素或免疫抑制剂治疗猝死。最终29例病情好转，13例长期维持性血透治疗，7例12个月内死亡，2例接受激素或免疫抑制剂治疗者病情被控制但死于合并重症感染。结论成人原发性 AASV 临床表现多样，以老年人为主，主要累及肺部和肾脏，ANCA 化验有特异性，糖皮质激素联合免疫抑制剂治疗有效。本病若在重要脏器损害前早诊断、早治疗，可降低病死率。
목적：탐토성년인원발성항중성립세포포장항체（ANCA）상관성소혈관염（AASV）적특점병분석기예후，이제고대본병적인식。방법선취원발성 AASV환자51례，대기림상표현、보조검사、병리결과、치료급예후등자료진행분석。결과51례중，폐부수루39례、신장수루40례；실험실검사수과양화물매（MPO）양성45례、단백매3（PR3）양성6례；12례행신장병리검사，표현위모세혈관반배사화신월체형성。51례환자중49례급여당피질격소치료（기중40례접수당피질격소연합면역억제제치료），1례부여면역억제제치료，1례미급접수격소혹면역억제제치료졸사。최종29례병정호전，13례장기유지성혈투치료，7례12개월내사망，2례접수격소혹면역억제제치료자병정피공제단사우합병중증감염。결론성인원발성 AASV 림상표현다양，이노년인위주，주요루급폐부화신장，ANCA 화험유특이성，당피질격소연합면역억제제치료유효。본병약재중요장기손해전조진단、조치료，가강저병사솔。
Objective To investigate the characteristics of antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis (AASV)in adults,and to analyze the prognosis for improving our understanding of the disease.Methods Clinical manifestations,laboratory exami-nations,pathological findings,treatment,prognosis and other data of 5 1 adult patients with prima-ry AASV were analyzed retrospectively.Results Among the 5 1 patients,lung involvement oc-curred in 39 and kidney involvement occurred in 40.Laboratory examinations showed positive re-sults for myeloperoxidase in 45 patients and for proteinase 3 in 6.Moreover,capillary loop necro-sis and crescent formation were found in the 1 2 patients who received renal pathological examina-tion.Among the 49 patients who received glucocorticoid treatment (40 received glucocorticoid and immunosuppressants,1 case were treated only with cyclophosphamide,1 did not receive hormones or immunosuppressants),2 9 had achieved improvement,1 3 were treated with long-term mainte-nance hemodialysis,7 died within 1 2 months,and 2 who received hormones or immunosuppressa-nts died of severe infection.Conclusion Primary AASV has diverse clinical manifestations in a-dults,especially in elderly patients.The Lung and kidney are the two organs most commonly in-volved.ANCA determination is specific and the combined treatment with glucocorticoid and im-munosuppressants is effective for AASV.Early diagnosis and treatment before organ damage can reduce the mortality in patients with AASV.