抗中性粒细胞胞质抗体相关性血管炎相关抗体研究进展

ANCA相关性血管炎(AAV)是一组累及多系统的寡免疫复合物型小血管炎,其确切病因及发病机制目前尚不明确。目前该病临床误诊率及病死率仍然较高。传统抗体ANCA仍是目前用于诊断及分型依据的特异性实验室指标,但其本身对于监测疾病的活动度尚缺乏足够的敏感度和特异度。因此发现并应用新型生物标记物对AAV进行诊断,监测疾病活动度,评估不同类型AAV的治疗效果以及预测其复发情况,有望实现AAV的精准诊断和治疗。     

抗中性粒细胞胞质抗体相关小血管炎误诊17例临床分析

报道误诊为其他疾病的17例抗中性粒细胞胞质抗体（ANCA）相关小血管炎（AASV）患者,其误诊原因主要为临床医师对AASV缺乏认识及警惕,没有详细系统地询问病史及既往史,患者首发表现缺乏特异性。常规开展ANCA、抗髓过氧化物酶抗体和抗蛋白酶3抗体检测,提高对AASV的认识及警惕性,有助于AASV的诊断。     

抗中性粒细胞胞质抗体相关性血管炎的治疗新进展

抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）在全球的发病率不断上升，迄今该病仍是预后差、造成多器官衰竭和威胁生命的疾病。近年，随着对该病发病机制研究的深入和新型免疫抑制剂的使用，患者的生存率得到了提高。本文仅就最近国内外有关AAV的治疗进展以及复发的防治做一简述。     

抗中性粒细胞胞质抗体相关性小血管炎的神经系统损害

目的 总结显微镜下多血管炎(MPA)、韦格纳肉芽肿病(WG)和Churg-Strauss综合征(CSS)患者的神经系统损害特点.方法 同顾性分析1991-2006年住院治疗的104例抗中性粒细胞胞质抗体(ANCA)相关性血管炎患者,其中MPA 58例,WG 31例,CSS 15例.总结患者的临床特征和神经系统损害特点.结果 患者诊断时平均年龄:MPA为50.7岁,WG为47.5岁,CSS为41.7岁.MPA组和WG组男女比例相似,但CSS组男性多见.MPA、WG和CSS组中分别有25例(43%),16例(52%)和13例(87%)出现周围或中枢神经损害,三组均以周围神经损害多见,但WG的颅神经受累明显高于其他两组.合并神经损害和无神经损害的患者血管炎活动指数(BVAC)在三组差异均无统计学意义.所有患者均进行了ANCA检测,MPA组中44例(76%)阳性,WG组中24例(77%)阳性,CSS组中4例(27%)阳性.结论 神经系统损害是ANCA相关性血管炎最常见的临床表现之一,风湿病医生应重视神经损害的诊断和及时治疗.     

抗溶酶体相关膜蛋白2抗体在抗中性粒细胞胞质抗体相关血管炎肾损害中的研究进展

抗中性粒细胞胞质抗体(ANCA)相关血管炎(AAV)是继发性肾损害的重要病因之一,发病率及致死率均较高,且病情易反复,治疗困难,其发病机制目前并不清楚。深入研究AAV致病原因及发病机制是提高疗效的重要方法,而抗溶酶体相关膜蛋白2(LAMP-2)抗体作为新发现的ANCA抗体,与AAV的关系成为目前研究的焦点之一。虽然目前研究结果并不一致,但随着细菌感染、Ⅰ型菌毛蛋白H(FimH)与LAMP-2分子模拟、动物实验及人体实验观察的深入,抗LAMP-2抗体将可能进一步揭示AAV肾损害发病机制,并可能成为AAV诊断、监测病情活动的新的标志物,为免疫抑制剂个体化治疗提供依据。本文就抗LAMP-2抗体与AAV肾损害的研究进展做一综述。     

抗甲状腺药物诱导的抗中性粒细胞胞质抗体相关性小血管炎

以丙基硫氧嘧啶(Propylthiouracil,PTU)、他巴唑(Thiamazole,MMI)等为代表的抗甲状腺药物(Antithyroid drugs,ATD)广泛应用于临床甲状腺功能亢进的治疗,同时也是导致药物相关性血管炎最常见的原因之一,其临床特点与原发性小血管炎相比有其特殊之处。本文回顾性分析国内外相关文献,对本病临床特点加以综述,总结其诊治进展,以期指导临床。     

抗中性粒细胞胞质抗体相关性血管炎的治疗现状及进展

随着大量针对抗中性粒细胞胞质抗体(ANCA)相关性血管炎(ANCA-associated systemic vasculitis, AASV)临床试验的开展,一些新的循证医学结果逐渐达成共识:(1)糖皮质激素和环磷酰胺仍是AASV诱导期最主要的治疗手段,静脉注射环磷酰胺较口服更好;(2)对早期轻症AASV,甲氨蝶呤可以替代环磷酰胺,重症患者可行血浆置换;(3)利妥昔单抗(美罗华)已被证明在诱导AASV缓解方面与环磷酰胺有同样效果,对复发病例更优,有望成为标准治疗药物;(4)维持期治疗可用硫唑嘌呤;(5)随着疾病机制的不断深入,“分子治疗”被寄予厚望.本文就近年来基于主要临床试验所取得的免疫治疗进展作一综述.     

利福平致抗中性粒细胞胞质抗体相关性小血管炎一例

抗中性粒细胞胞质抗体(ANCA)相关性小血管炎是一类系统性自身免疫性疾病,分为原发性和继发性.临床上利福平致ANCA相关性小血管炎较少见,现将我科近期诊治的1例报告如下.     

抗中性粒细胞胞质抗体相关性小血管炎发病机制的研究进展

抗中性粒细胞胞质抗体相关性小血管炎是指以微小动脉、毛细血管、小静脉等小血管管壁的炎症和纤维素样坏死为病理特征的一组系统性疾病。目前发现的原发性小血管炎主要有以下几类：韦格纳肉芽肿病（WG）、显微镜下多血管炎（MPA）、变应性肉芽肿性血管炎（CSS）、节段坏死性新月体性肾炎（NCGN）。而抗中性粒细胞胞浆抗体（ANCA）则是一种以中性粒细胞和单核细胞胞浆成分为靶抗原的自身抗体。     

利福平诱发抗中性粒细胞胞质抗体阳性小血管炎2例

抗中性粒细胞胞质抗体（anti-neutrophil cytoplasmic antibody ANCA）是一组以中性粒细胞、单核细胞胞质为靶抗原的抗体谱,可作为某些原发性小血管炎的标志抗体.     

核周型抗中性粒细胞胞浆抗体在溃疡性结肠炎诊断中的意义

目的了解血清中核周型抗中性粒细胞胞浆抗体(pANCA)对溃疡性结肠炎诊断的敏感性及特异性。方法本文采用间接免疫荧光法对我院51例溃疡性结肠炎、15例克罗恩病、30例对照患者血清中核周型抗中性粒细胞胞浆抗体进行检测。结果 51例溃疡性结肠炎患者中32例核周型抗中性粒细胞胞浆抗体阳性,其敏感性及特异性分别为62.75%及95.56%。15例克罗恩病患者中有2例阳性核周型抗中性粒细胞胞浆抗体阳性,阳性率为13.33%,30例对照组未发现核周型抗中性粒细胞胞浆抗体阳性患者,组间差异具有统计学意义(P<0.05)。结论血清中抗中性粒细胞胞浆抗体对溃疡性结肠炎具有一定的敏感性及高度的特异性,对其检测有利于溃疡性结肠炎的诊断及鉴别诊断。     

Randomized controlled trials in vasculitis associated with anti-neutrophil cytoplasmic antibodies

PURPOSE OF REVIEW: To review three major randomized clinical trials in forms of vasculitis associated with anti-neutrophil cytoplasmic antibodies. RECENT FINDINGS: The design features, results, and context of the Cyclophosphamide versus Azathioprine for the Remission Phase of Vasculitis, Non-renal AAV alternatively treated with Methotrexate, and Wegener Granulomatosis Etanercept Trial are reviewed. SUMMARY: Until recently, therapies for Wegener granulomatosis and microscopic polyangiitis have been based primarily on a relatively small number of nonrandomized studies. During the past 18 months, three randomized, controlled trials (one double-blinded) have been performed in vasculitis associated with anti-neutrophil cytoplasmic antibodies. Careful comparisons of the results of these trials yield insights into new standards of care for vasculitis associated with anti-neutrophil cytoplasmic antibodies. This paper summarizes the designs of these three trials; highlights their principal conclusions, strengths, and shortcomings; and distills from their results several recommendations on major questions related to the therapy of vasculitis associated with anti-neutrophil cytoplasmic antibodies.     

Pitfalls with anti-neutrophil cytoplasmic antibodies (ANCA)

Summary A 39-year-old Ugandan student is described presenting with general malaise, fever and a pulmonary infiltrate. Open lung biopsy showing infarction and positive ANCA lead to a diagnosis of Wegener's granulomatosis and a treatment with immunosuppressive drugs was instituted. Five weeks after admission, however, sputum cultures turned out to be positive for Mycobacterium tuberculosis. The importance of ANCAinterpretation and the possibility of false positive results is discussed.     

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies in patients with inflammatory bowel disease

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies (x-, c- and pANCA, respectively) are associated with a variety of inflammatory diseases, including inflammatory bowel disease (IBD). Anti-neutrophil cytoplasmic antibodies are more common in patients with ulcerative colitis (UC) than in patients with Crohn's disease (CD). Most publications only refer to p- and cANCA in relation to IBD. We have prospectively evaluated the reactivity of sera from 58 patients with IBD and 10 healthy controls against human neutrophils with emphasis on the distinction of the ANCA types. The sera were incubated with ethanol- and formaldehyde-fixed granulocytes to differentiate between c-, p- and xANCA. The results showed that 10 of 24 patients with UC were positive for ANCA, whereas only one of 34 patients with CD was ANCA positive. These results correspond to a sensitivity of 42%, a specificity of 97%, a negative predictive value of 91% and a positive predictive value of 75% in UC. Of the 11 ANCA-positive sera, two showed a cytoplasmic staining pattern, three showed a perinuclear and six an atypical staining pattern. The disease activity was not correlated to either the ANCA titre or to the presence of ANCA in the serum. In conclusion, ANCA are of limited value in differentiating between UC and CD. Because the majority of ANCA in patients with IBD are xANCA, these ANCA should be explored by not only incubating on ethanol-fixed granulocytes, but also on formaldehyde-fixed granulocytes.     

抗中性粒细胞胞浆抗体相关性小血管炎1例并文献复习

抗中性粒细胞胞浆抗体（anti-neutrophil cytoplasmic antibody,ANCA）相关性小血管炎（ANCA associated systemic vasculitis,AASV）是一组以ANCA阳性为突出临床特征,累及多个系统的血管炎性疾病。因其临床表现缺乏特异性,易被漏诊、误诊。现将我科收治的1例以呕吐为首发表现的AASV患者的临床特点进行总结和分析。     

抗中性粒细胞胞浆抗体相关小血管炎肾损伤并发消化道大出血病例报告并文献复习

目的:探讨抗中性粒细胞胞浆抗体(ANCA)相关小血管炎肾损伤并发消化道大出血的临床特点及其治疗方法。方法:回顾性分析1例ANCA相关血管炎肾损害并发消化道大出血患者的临床资料并复习文献。结果:患者在应用激素及免疫抑制剂治疗过程中出现消化道大出血,行部分坏死肠管切除及组织学检查后,证实为ANCA相关小血管炎(韦格纳肉芽肿);术后予以血浆置换并应用激素治疗原发病,患者平稳度过危险期,病情逐渐好转。结论:ANCA相关小血管炎可以导致严重消化道大出血,明确诊断和内、外科协同诊治可提高抢救成功率。