狼疮性肾炎患者血清抗中性粒细胞胞浆抗体的检测及意义

系统性红斑狼疮(SLE)是我国最常见的自身免疫性疾病之一,狼疮性肾炎为其较常见的组织损伤,患者血清中含有多种自身抗体,除既往研究较多的抗核抗体(ANA)外,近年来还开展了抗中性粒细胞胞浆抗体(ANCA)的研究.本文通过对30例狼疮性肾炎(LN)患者血清中ANCA检测,旨在探讨ANCA在狼疮性肾炎中的阳性率以及与狼疮性肾炎的关系.     

儿童抗中性粒细胞抗体相关性血管炎2例报告并文献复习

抗中性粒细胞抗体(ANCA)相关性血管炎(AASV)是一类由遗传、环境及免疫等诸多因素共同作用所致的系统性自身免疫性疾病,主要累及中小血管,实验室检查ANCA常阳性。主要包括韦格纳肉芽肿病(WG)、显微镜下型多血管炎(MPA)和变应性肉芽肿性血管炎(CSS),常累及全身多脏器。由于临床表现复杂,易导致误诊、漏诊。多见于中老年人,儿童AASV十分少见。笔     

ANCA相关性系统性小血管炎发病机制的研究进展

<正>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)是一种严重的累及小和中等血管引起严重的全身多系统器官功能衰竭、血清中存在针对靶抗原蛋白酶3(PR3)或髓过氧化物(MPO)的ANCA阳性的自身免疫性疾病,它以小血管壁的炎症和纤维素样坏死为主要特征。主要     

抗中性粒细胞胞浆抗体相关性疾病及系统损害

抗中性粒细胞胞浆抗体(ANCA)作为判定血管炎的重要标记物[1]已逐渐被认识,但其具体靶抗原抗体的相关性疾病和出现的系统性损害认识还不够全面,下面给予进一步总结明确。     

尿毒症新发ANCA相关性血管炎的诊治

抗中性粒细胞胞浆抗体(antineutrophil cyto-plasmic antibody,ANCA)相关性系统性血管炎(ANCA-associated systemic vasculitis,AASV)是一组以小血管壁炎症和纤维素样坏死为特征、抗中性粒细胞胞浆抗体阳性的自身免疫性疾病[1],主要包括显微镜下多血管炎(MPA)、韦格纳肉芽肿(WG)、变应性肉芽肿性血管炎(CSS)三种.本病可累及全身多个脏器,以肾、肺累及最为常见,临床上一些患者因诊治不及时而进入终末期肾脏病(end-stage renal disease,ESRD)需长期肾替代治疗.     

酶联免疫吸附试验检测血清抗中性粒细胞胞质抗体方法的建立与临床应用

目的建立总抗原酶联免疫吸附试验(ELISA)检测血清抗中性粒细胞胞质抗体(ANCA)的方法,初步探讨其在ANCA相关血管炎中的表达及临床应用价值。方法从人的中性粒细胞中提取总抗原,用棋盘滴定法选择ELISA测定的最佳条件,确定临界值。测定系统性红斑狼疮(SLE)、肾病、血管炎、类风湿关节炎(RA)等患者血清总ANCA,并与间接免疫荧光(IIF)法、特异性髓过氧化物酶(MPO)-ANCA及蛋白酶3(PR3)-ANCA进行分析比较。结果总抗原ELISA与IIF法检测肾病、SLE、血管炎及RA患者血清ANCA阳性率比较差异均无统计学意义(P>0.05)。抗原特异性ELISA、IIF法检测肾病、血管炎、RA患者血清ANCA阳性率比较差异均无统计学意义(P>0.05),而SLE差异有统计学意义(P<0.05)。结论ELISA检测ANCA可行且具有广泛的推广应用前景。     

甲巯咪唑诱发抗中性粒细胞胞浆抗体相关性血管炎伴肾损害1例

目的提示临床重视抗甲状腺药物甲巯咪唑具有诱发抗中性粒细胞胞浆抗体(ANCA)相关性血管炎的不良反应。方法回顾分析1例确诊的因长期服用甲巯咪唑而诱发ANCA相关性血管炎并伴发肾功能损伤的病例。结果患者因ANCA相关性血管炎伴肾功能损伤症状入院。综合分析患者症状体征、检验结果及相关既往史、用药史,考虑为药物性ANCA相关性血管炎,故停用可疑药物并予对症治疗,最终患者ANCA转阴,肾功能恢复正常。结论抗甲状腺药物甲巯咪唑可诱发ANCA相关性血管炎,临床运用该药物需重视检测相关指标。     

ANCA相关性血管炎的临床分析(附51例报告)

抗中性粒细胞胞浆抗体相关性血管炎(AAV)是以血管壁炎症和纤维素样坏死为病理特点,可累及全身多器官的一种自身免疫性疾病,主要包括显微镜下多血管炎(MPA)、肉芽肿性多血管炎(GPA)和嗜酸性肉芽肿性多血管炎.由于AAV发病率低和临床表现复杂多变导致早期、及时的诊断该病是比较困难的.目前,抗中性粒细胞胞浆抗体(ANCA)验室检测较为普遍,提高了AAV的诊断,但大多患者就诊时常病情严重,为提高对该病的认识、诊断和预后判断,现将我院门诊及住院诊治病例回顾性分析,总结如下.     

ANCA检测在临床中的应用

抗中性粒细胞胞浆抗体（antineutrophil cytoplasmic antibody，ANCA）是针对中性粒细胞颗粒成分和单核细胞溶酶体的自身抗体，作为韦格纳肉芽肿（Wegener＇s granulomatosis，WG）为代表的系统性坏死性小血管炎的血清学标志性抗体已得到国际广泛认同，并对其病情缓解、复发具有一定的预测价值。近年来ANCA的实验室检测技术突飞猛进，在系统性红斑狼疮（systemic lupus erythematosus，SLE）、类风湿关节炎（rheumatoid arthritis，RA）等许多结缔组织病（connective tissue disease，CTD）患者血清中陆续检测到ANCA。     

抗中性粒细胞胞浆抗体在诊断系统性红斑狼疮活动中的临床意义

目的 探讨抗中性粒细胞胞浆抗体(ANCA)在系统性红斑狼疮(SLE)活动中的临床意义.方法 用间接免疫荧光法(IIF)及免疫印迹法,检测97例SLE患者血清中的ANCA、抗ds-DNA抗体、多种自身抗体及补体.综合分析SLE活动中的发热、关节肿痛、皮疹、肾损害、肺损害等临床表现.结果 (1)97例SLE患者中26例ANCA阳性,均为核周型(pANCA),阳性率为26.80%.(2)26例pANCA阳性组活动期20例(76.92%);71例阴性组活动期32例(45.07%),P＜0.01.(3)pANCA阳性组SLE活动期的实验室指标及临床表现均高于阴性组P＜0.05,差异均有统计学意义.结论 pANCA阳性对判断SLE活动性具有较明确的临床意义.     

抗中性粒细胞胞质抗体相关性血管炎的生物治疗

抗中性粒细胞胞质抗体(ANCA)相关性血管炎包括韦格纳肉芽肿(WG)、显微镜下多血管炎(MPA)和变应性肉芽肿性血管炎,以中、小血管受累为主.包括环磷酰胺在内的免疫抑制荆与糖皮质激素的联合治疗,能明显提高患者的生存率,但如何使难治性ANCA相关性血管炎缓解以及如何在缓解后减少其复发,仍然是治疗的难点.生物制剂除了在类风湿关节炎等炎症病变中得到成功应用,近年来,一些学者应用生物制剂如肿瘤坏死因子拮抗剂、抗B细胞抗体治疗ANCA相关性血管炎取得了一定的临床疗效.     

显微镜多血管炎5例分析

目的　提高对显微镜多血管炎临床特征的认识。方法　报道 5例显微镜多血管炎 ,并进行文献复习。结果　 4例以发热 ,3例以关节肿痛 ,1例以腹痛、腹泻和胃肠道出血为起病症状。 5例都有肾损害 ,3例肺受累。 5例血沉都增快 ,C反应蛋白增高 ,有不同程度贫血和低蛋白血症 ,补体都正常 ,核周型 抗中性粒细胞胞质抗体 (p ANCA)和髓过氧化物酶 抗中性粒细胞胞质抗体 (MPO ANCA)都阳性。结论　不明原因的长期发热、关节肿痛伴肺肾受累 (尤其肾功能急剧恶化的肾小球肾炎而无肾性高血压 ,不明原因的咯血 ) ,补体正常 ,p ANCA和MPO ANCA阳性 ,排除继发性小血管炎 ,应诊断显微镜多血管炎     

Respiratory system involvement in antineutrophil cytoplasmic-associated systemic vasculitides: clinical, pathological, radiological and therapeutic considerations

Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg- Strauss syndrome (CSS) are small-vessel vasculitides that, because of their frequent association with antineutrophil cytoplasmic antibodies (ANCA), are usually referred to as ANCA-associated systemic vasculitides (AASV). The diagnosis of AASV is made on the basis of clinical findings, biopsy of an involved organ and the presence of ANCA in the serum. Lung disease is a very common and important clinical feature of AASV. In WG, almost all patients have either upper airway or lower respiratory tract disease. Solitary or multiple nodules, frequently cavitated, and masses are the most common findings on chest radiography. Asthma is a cardinal symptom of CSS, often preceded by allergic rhinitis. Pulmonary transient and patchy alveolar infiltrates are the most common radiographic findings. In MPA, diffuse alveolar haemorrhage as a result of alveolar capillaritis is the most frequent manifestation of respiratory involvement, and is clinically expressed as haemoptysis, respiratory distress and anaemia. However, diffuse alveolar haemorrhage may also be subclinical and should be suspected when a chest radiograph demonstrates new unexplained bilateral alveolar infiltrates in the context of falling haemoglobin levels. Normal and high-resolution CT have a higher sensitivity than chest radiography for demonstrating airway, parenchymal and pleural lesions. However, many of these radiological findings are nonspecific and, therefore, their interpretation must take into account all clinical, laboratory and pathological data. Therapy of AASV is commonly divided into two phases: an initial 'remission induction' phase, in which more intensive immunosuppressant therapy is used to control disease activity, and a 'maintenance' phase, which uses less intensive therapy, for maintaining disease remission while lowering the risk of adverse effects of immunosuppressant drugs. In patients with AASV refractory to standard therapy with corticosteroids and oral cyclophosphamide, new therapeutic options are now available. Recurrence of pulmonary symptoms suggesting a flare indicates the need for a careful search for an opportunistic lung infection or iatrogenic pulmonary complications. In conclusion, involvement of the respiratory system is a very common and important organ manifestation of AASV. Respiratory system involvement comprises a wide spectrum of clinical features and radiological findings, and because of its frequency and prognostic significance, a complete assessment of the respiratory system should be included in the work-up of all patients with AASV.     

Biological drugs in ANCA-associated vasculitis

The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) constitute a wide spectrum of clinical manifestations of systemic vasculitis which may range from limited disease to organ or life-threatening disease. The introduction of biologics for the management of severe ANCA-associated vasculitis and severe, relapsing disease refractory to conventional immunosuppressants, has significantly improved the clinical prognosis of these autoimmune disorders. Rituximab, an anti-CD20 monoclonal antibody is licenced for remission induction in severe GPA and MPA and the management of severe relapsing, refractory GPA and MPA. Belimumab, an anti-B lymphocyte stimulatory monoclonal antibody is in clinical trials for the management of the ANCA-associated vasculitis GPA. Mepolizumab and Omalizumab are biologics which have been reported to be efficacious in refractory asthma associated with EGPA. The role of anti-TNF therapy and T cell targeting drugs in ANCA-associated vasculitis is less clear due to limited study data. This review will summarise the clinical trials and clinical practice use of biologic treatment strategies for the management of ANCA-associated vasculitis.     

Further studies on the therapy of organophosphorous anti-cholinsterase intoxication with veratrinic compounds; The role of calcium

Experiments were carried out to investigate the role of calcium in the therapy of soman intoxication with 9-anthroic acid (ANCA), a compound with veratrine-like pharmacological properties. The effects of ANCA on the respiratory paralysis and on the calcium content of the blood and that of the hindleg muscles were determined in anaesthetized, atropinized rats injected with 4 × LD₅₀ soman. The respiratory paralysis which in control animals occurs within a few min after the injection of soman can be delayed about 2.5 hr hy treatment with ANCA. It was found that ANCA causes a small decreases of the blood calcium content, an effect which is potentiated by soman. A comparison was made between the calcium accumulation in the indirectly stimulated gastrocnemius-soleus muscles in these animals with that in the non-stimulated muscles on the other side.Whereas the injection of soman or ANCA alone caused no change, the combination of the two drugs induced a two-fold increase in the accumulation of calcium in the stimulated muscles. The non-stimulated muscles remained unaffected. Teh accumulation of calcium in the stimulated muscles induced by soman and ANCA could be partly antagonized by lowering the free calcium concentration of the blood by EDTA. Moreover, treatment with EDTA improved the therapeutic effects of ANCA. It is concluded that the therapy of soman poisoning with ANCA falls short in completely preventing respiratory failure since ANCA causes an accumulation of calcium in the stimulated muscles of soman-poisoned animals.     

系统性红斑狼疮活动期与其伴新月体肾炎患者抗中性粒细胞胞浆抗体检测的临床意义

目的探讨抗中性粒细胞胞浆抗体（ANCA）在系统性红斑狼疮（SLE）活动期和在狼疮伴新月体肾炎诊断中的意义。方法采用间接免疫荧光（IIF）法和免疫印迹法分别测定132例SLE患者,其中59例为SLE稳定期、73例为SLE活动期患者的ANCA和SLE活动期相关自身抗体：抗ds—DNA抗体、AnuA和His,分析ANCA与SLE活动期相关自身抗体和狼疮疾病活动指数之间的关系,同时分析ANCA阳性的SLE患者伴新月体肾炎的发病率。结果ANCA在SLE活动期的阳性率为30．1％,且均为PANCA,并显著高于SLE稳定期（P〈0．05）。ANCA在SLE活动期阳性率与SLE活动期相关自身抗体dsDNA、AnuA和His的阳性率呈成正相关。ANCA阳性患者中的SLE活动指数（SLEDAI）与ANCA阴性组相比,差异有统计学意义（P〈0．05）。在ANCA阳性的29例SLE患者中,病理切片显示有17例伴新月体肾炎（58．6％）,103例ANCA阴性患者中,伴新月体肾炎20例（19．4％）,ANCA阳性组中伴新月体肾炎发病率明显高于阴性组（P〈0．01）。结论ANCA对判断狼疮活动性有一定的临床应用价值,对诊断SLE伴新月体肾炎有较为重要的临床意义。     

甲巯咪唑致粒细胞缺乏合并ANCA阳性1例并文献复习

抗甲状腺药物是Graves’病的一线治疗药物,粒细胞缺乏是其少见但严重的副作用,发生机制仍不十分清楚,目前认为主要和免疫反应有关。PTU能诱导ANCA的产生及ANCA相关性血管炎的发生,部分患者合并出现粒细胞减少。虽然粒细胞减少症并未列入ANCA相关的疾病谱,近年越来越多文献报道支持ANCA参与粒细胞减少的发生。这里报道一个MMI治疗后出现ANCA阳性合并严重粒细胞减少不伴血管炎的病例,并对相关的文献进行复习。     

抗中性粒细胞胞浆抗体及相关实验室指标在肺纤维化患者中的应用探讨#br#

摘要：目的　探讨肺纤维化（PF）患者抗中性粒细胞胞浆抗体（ANCA）阳性率、核型和靶抗原情况及其与常见临床指标的关系。方法　选取诊断为PF且同时行ANCA检测的患者302例。其中72例ANCA阳性PF患者为ANCA阳性PF组；230例ANCA阴性PF患者根据随机数字表法抽取72例为ANCA阴性PF组。另外，选取25例不伴PF的ANCA阳性患者作为ANCA阳性非PF组，20例健康体检者作为健康对照组。分析各组的ANCA核型及靶抗原、炎症、凝血-纤溶、免疫功能、肾功能相关实验室指标。结果　ANCA阳性PF患者占同期PF患者的23.84%。ANCA阳性PF组核周型ANCA（pANCA）阳性比例较胞浆型ANCA（cANCA）更多，靶抗原髓过氧化物酶（MPO）阳性比例较蛋白酶3（PR3）更多。ANCA阳性PF组C反应蛋白（CRP）较ANCA阴性PF组显著升高，ANCA阳性PF组、ANCA阴性PF组、ANCA阳性非PF组的血沉（ESR）、CRP、降钙素原（PCT）、D二聚体（D-Dimer）、纤维蛋白原（FIB）较健康对照组显著升高（P＜0.008）。ANCA阳性PF组和ANCA阳性非PF组补体3（C3）较健康对照组和ANCA阴性PF组显著降低，ANCA阳性非PF组的血肌酐（Scr）较ANCA阴性PF组显著升高（P＜0.008）。结论　诊断PF时应考虑存在抗中性粒细胞胞浆抗体相关血管炎的可能，同时应重视ESR、CRP、PCT、D-dimer、FIB、C3和Scr指标的变化。     

抗中性粒细胞胞浆抗体水平检测对血管炎的诊断价值

目的：研究检测抗中性粒细胞浆抗体（ANCA）水平对血管炎的诊断价值。方法：用间接免疫荧光法（IIF）检测125例患者及30例健康成人血清ANCA水平，并以酶联免疫吸附法（ELISA）检测抗人髓过氧化物酶（MPO）抗体，结果：韦格纳氏肉芽肿（WG）患者ANCA阳性率为77．78％，明显高于正常对照组及其它疾病组（P＜0．001，P＜0．01），MPO是核周型ANCA（P－ANCA）的主要抗原。活动期WG患者胞浆型ANCA（C－ANCA）呈阳性，且滴度增高；恢复期C－ANCA转阴，滴度明显下降，结论：C－ANCA检测对WG诊断具有较高特异性，C－ANCA可作为判断WG活动性的指标之一；P－ANCA无特异性，但与血管炎有关。     

Clinical overview of vasculitic syndromes in the pediatric age

Vasculitic syndromes comprise a heterogeneous group of disorders sharing the hystopathologic features of inflammation and necrosis in blood vessels. Their clinical expression depends on site, type and size of the involved vessels and severity of the associated inflammatory symptoms. Classification of vasculitides based on the size of the affected vessels is the most widely used in children. Many different vasculitides with indistinguishable clinical presentation have very different prognosis and treatments. Among the primary systemic non-granulomatous vasculitides of medium-sized vessels in pediatrics we have to consider Kawasaki disease and among the small-sized ones Henoch-Sch?nlein purpura, which is the most frequent vasculitis of the pediatric age and is characterized by vascular deposition of IgA-dominant immune complexes. Accurate diagnosis is the mainstay for the definition of the best therapeutical proposal, though therapies available result largely empirical and based on trials with limited numbers of pediatric patients.