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.     

系统性血管炎中抗内皮细胞抗体的检测及其临床意义分析

目的研究抗内皮细胞抗体（AECA）在系统性血管炎中阳性率,并分析其临床意义.方法固定的人脐静脉内皮细胞（HUVEC）为抗原,细胞酶联免疫吸附试验（ELISA）法检测129例系统性血管炎,57例系统性红斑狼疮（SLE）、25例类风湿关节炎（RA）等患者和85名正常对照者AECA阳性率并分析系统性血管炎组AECA与临床生化指标的相关性.结果系统性血管炎（58.9%）和SLE（46%）患者的AECA阳性率明显高于RA（4%）和正常人组（2.4%）（P＜0.01）.AECA在不同系统性血管炎中阳性率分别为：白塞病（BD）48%（28/59）、大动脉炎（TA）79%（22/28）、韦格纳肉芽肿病（WG）65%（13/20）、结节性多动脉炎（PAN）63%（5/8）、显微镜下多血管炎（MPA）44%（4/9）、变应性肉芽肿血管炎（CSS）80%（4/5）.AECA阳性的TA患者的血沉（ESR）平均水平明显高于AECA阴性组（P＜0.05）.PAN、WG、MPA、CSS患者中,AECA阳性者ESR水平（P＜0.05）和伯明翰系统性血管炎临床活动评分标准（BVAS）值（P＜0.01）明显高于AECA阴性者,BD患者中,AECA阳性病人具有皮肤针刺反应阳性率明显增多（P＜0.05）.随诊的11例系统性血管炎患者治疗前后AECA滴度和ESR的变化,差异有统计学意义（P＜0.05）.结论不同的系统性血管炎和SLE患者血清中均有较高的AECA阳性率,且此抗体与系统性血管炎的临床活动度明显相关,可作为观察病情活动的指标之一.     

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。     

白细胞介素-8受体A型和B型在强直性脊柱炎中的表达及其意义探讨

目的 检测白细胞介素-8受体A型（CXCR1）和白细胞介素-8受体B型（CXCR2）在强直性脊柱炎（AS）患者外周血中性粒细胞、CD14^＋单核细胞和CD3^＋T细胞上的表达水平，探讨其与AS疾病活动的相关性和可能涉及的AS炎症发病机制。方法 研究对象包括30例活动期AS患者，30例活动期类风湿关节炎（RA）患者和30名健康对照者，应用流式细胞术（FCM）检测CXCR1、CXCR2分别在AS患者、RA患者和健康对照者外周血中性粒细胞、CD14^＋单核细胞和CD3^＋T细胞上平均荧光强度（MFI）的表达水平，并和AS患者的临床BASFI、BASDAI、红细胞沉降率（ESR）、血清C反应蛋白（CRP）等指标进行相关分析。结果 CXCR1在AS患者组外周血CD3^＋T细胞上MFI表达水平（41±24）分别较RA患者组（18±10）和健康对照组（19±7）高（P均〈0．01）。CXCR2在AS患者组外周血CD14^＋单核细胞MFI表达水平（210±54）较健康对照组（300±52）低（P〈0．01），与RA患者组（191±53）比较差异无统计学意义（P〉0．05）；CXCR2在AS患者外周血CD14^＋单核细胞上MFI表达下降与患者毕氏疾病功能指数（BASFI）（r=-0．394，P=0．031）、毕氏AS疾病活动指数（BASDAI）（rs=-0．378，P=0．040）、ESR（rs=-0．465，P=0.010）、CRP（rs=-0．648．P=0．000）存在着负相关关系。结论 CXCR1和CXCR2分别在AS患者外周血CD3^＋T细胞和CD14^＋单核细胞表达异常，提示它们可能参与了AS的发病过程。检测AS患者外周血CD14^＋单核细胞的MFI表达水平可能是评价AS疾病活动性有价值的潜在的生物学标志之一。     

抗中性粒细胞胞浆抗体相关性血管炎的药物治疗进展

原发性小血管炎是一类以小动脉、小静脉、毛细血管等发生血管壁炎症、纤维素样坏死为病理特征的一组自身免疫性疾病。在原发性小血管炎中,部分疾病的发生与抗中性粒细胞胞浆抗体（ANCA）密切相关。因此,称之为ANCA相关性血管炎（AAV）。AAV主要包括韦格纳肉芽肿（WG）、显微镜下多血管炎（MPA）、变应性肉芽肿性血管炎（CSS）和局限于肾脏的血管炎（RLV）。     

系统性血管炎患者血浆可溶性CD146、肿瘤坏死因子-α水平变化的临床研究

目的 观察系统性血管炎患者治疗前后血浆中可溶性CD146（sCD146）、肿瘤坏死因子-α （TNF-α）水平的动态变化,探讨其在系统性血管炎发病机制中的意义.方法 60例系统性血管炎患者为观察组,30例健康体检者为对照组.酶联免疫吸附试验（ELISA）检测患者血浆中sCD146、TNF-α水平,并分析其与系统性血管炎患者的病程、年龄、红细胞沉降率（ESR）、C反应蛋白（CRP）、血肌酐（sCr）、补体（CH50）、伯明翰血管炎活动指数（BVAS）的相关性.结果 观察组血浆sCD146、TNF-α水平明显高于对照组（P＜0.01）;观察组治疗后（好转期）血浆sCD146水平明显高于治疗前（活动期）（P＜0.01）.观察组治疗前sCD146与年龄、sCr水平呈明显正相关（P＜0.05）.结论 sCD146可作为系统性血管炎临床诊断的标志物之一,其产生并非主要由TNF-α调节.     

白细胞黏附分子CD54、CD62L、CD44在慢性阻塞性肺疾病患者的变化

目的 探讨白细胞黏附分子CD54、CD62L、CD44在慢性阻塞性肺疾病（chronic obstructive pulmonary disease,COPD）发生中的作用。方法 采用流式细胞术,选用单克隆抗体检测COPD患者40例（A组：A1组急性加重期20例,A2组经治疗后进入稳定期20例）外周血白细胞（中性粒细胞、淋巴细胞、单核细胞）表面CD54、CD62L及CD44表达,并设立健康人对照组（B组）20例。结果 A1组中性粒细胞（N）、淋巴细胞（L）和单核细胞（M）CD54阳性表达率较B组显著升高（P〈0．05）;A1组NCD62L阳性表达率较B组显著降低（P〈0．05）;A1组L、MCD62L及N、L、MCD44阳性表达率与B组间亦均无显著性差异（P〉0．05）。A2组NCD62L阳性表达率较B组显著降低（P〈0．05）;A2组LCD62L和CD44阳性表达率较B组显著升高（P〈0．05）;A2组N、L、MCD54,MCD62L及N、MCD44阳性表达率与B组间亦均无显著性差异（P〉0．05）。A1组NCD54阳性表达率较A2组显著升高（P〈0．01）;A2组LCD62L阳性表达率较A1组显著升高（P〈0．01）;A1组L、McD54,N、MCD62L及N、L、MCD44阳性表达率与A2组间无显著性差异（P〉0．05）。结论 COPD急性加重期患者通过外周血中性粒细胞、淋巴细胞、单核细胞表面CD54表达上调,COPD稳定期患者通过外周血淋巴细胞表面CD62L和CD44表达的上调,参与COPD的病理生理过程。     

系统性红斑狼疮骨髓T细胞活化及其与病情活动的相关性分析

目的 探讨系统性红斑狼疮（SLE）患者骨髓T淋巴细胞的活化状态及多种活化的T细胞亚群与疾病活动指标之间的相关性。方法 SLE患者11例（其中活动期6例，非活动期5例）和健康对照8名。应用流式细胞术比较活动期和非活动期SLE患者骨髓T细胞CD25、人类白细胞抗原（HLA）-DR的表达。用直线相关分析活化T细胞亚群与SLE疾病活动性评分（SLEDAI）、尿蛋白定量（24h）、血清补体C3、C4的相关性。结果 活动期SLE、非活动期SLE与正常对照相比骨髓T细胞CD25、HLA—DR的表达，差异均无统计学意义（P〉0．05）。活动期SLE骨髓T细胞HIJA—DR^＋、CD4^＋HLA—DR^＋、CD8^＋HLA—DR^＋的表达均明显高于正常对照外周血（P〈0．05）。11例SLE患者骨髓CD3^＋HLA—DR^＋、CD4^＋HLA—DR^＋细胞与C3之间均呈显著负相关（r=-0．682，r=-0．675，P均〈0．05）；CD3^＋HLA—DR^＋、CD8^＋-HLA—DR^＋细胞与尿蛋白定量（24h）之间均呈显著正相关（r=-0．712，r=-0．688，P均〈0．05）。活动期SLE骨髓CD3^＋HLA—DR^＋、CD8^＋HLA—DR^＋细胞与C3之间均呈显著负相关（r=-0．943，r=-0．829，P均〈0．05）。结论 活动期SLE患者骨髓T细胞活化明显高于正常对照外周血，且骨髓中活化的T细胞与疾病活动指标相关。     

抗中性粒细胞胞质抗体对中性粒细胞表面FcγⅡ/Ⅲ受体表达的影响及其意义

目的 探讨抗中性粒细胞胞质抗体(ANCA)对中性粒细胞表面FcγⅡ/Ⅲ(CD32/CD16)受体表达的影响及临床意义.方法 提纯活动期ANCA相关性小血管炎(AASV)患者的ANCA IgG,分离健康人新鲜中性粒细胞,以ANCA刺激粒细胞1 h后,流式细胞仪检测CD32/CD16的表达.流式细胞仪检测18例系统性小血管炎患者CD32/CD16的表达,以35名健康人为对照.同时对18例患者临床表现及血管炎活动情况进行评分,与CD32/CDl6表达进行相关分析.结果 ANCA IgG与正常人IgG相比显著上调中性粒细胞表面CD16的表达(Mnx 67±23 vs 54±21,P<0.01),但对CD32的表达无影响(Mnx 21±8vs 16±8,P>0.05).系统性小血管炎患者CD16表达的平均荧光强度显著高于正常人(Mnx 62±12 vs 53±10.P<0.01),而CD32的表达与正常人差异无统计学意义.CD16表达与伯明翰小血管炎呈显著正相关(P<0.01).结论 ANCA相关性小血管炎患者高表达Fcγ受体,ANCA可以干预CD32/CD16受体的表达,调控FCγ受体表达可能是系统性小血管炎的发病机制之一,监测CD16的表达水平对于判断疾病活动具参考价值.     

25例肺部受累的原发性小血管炎的HRCT表现及临床分析

目的研究肺部受累的原发性小血管炎的HRCT表现等临床特点。方法对本院2004年1月~2010年1月收治的肺部受累的19例显微镜下多血管炎（MPA）患者及6例变应性肉芽肿性血管炎（CSS）患者的临床资料进行回顾性分析。结果 19例MPA的肺部HRCT表现有：（1）两肺弥漫浸润性阴影、类似肺间质性改变12例;（2）肺内多发斑片影7例。6例CSS的肺部HRCT均表现为多发斑片影。19例MPA中抗中性粒细胞胞浆抗体（ANCA）阳性18例,阳性率高达94.74%。6例CSS中ANCA阳性1例。结论肺部受累的原发性小血管炎HRCT影像学表现无明显特异性,结合临床及实验室检查,可以提高认识,早期诊治。     

Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden

OBJECTIVES: To estimate the point prevalence (p.p.) of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) within a defined population in southern Sweden. METHOD: A cross-sectional p.p. study using multiple sources for case identification. The study area, a healthcare district around the city of Lund in southern Sweden (Mellersta Sk?nes sjukv?rdsdistrikt), had, on 31 December 2002, a total population of 287 479 inhabitants. All the identified cases were verified by medical record review. The patients were classified according to an algorithm based on the American College of Rheumatology classification criteria 1990 and the Chapel Hill Consensus Conference definitions 1994. RESULTS: Eighty-six patients (49% female) with a median age of 64.8 yrs (range 15-90.5) fulfilled the study criteria. There were 46 patients with WG; 27 with MPA; nine with PAN; and four with CSS. The p.p. per million inhabitants was estimated on 1 January 2003 to be 160 (95% confidence interval 114-206) for WG, 94 (58-129) for MPA, 31 (11-52) for PAN and 14 (0.3-27) for CSS. Capture-recapture analysis estimated the completeness of the case finding to 96%. CONCLUSIONS: The prevalence of WG, MPA, PAN and CSS in our district is the highest figure reported so far. Explanations for this finding may include high incidence, extensive ANCA-testing, good survival as well as sensitive search methods for case identification.     

Detection of coronary artery lesions and myocardial necrosis by magnetic resonance in systemic necrotizing vasculitides

Objective

Myocardium and coronary arteries can occasionally be affected in patients with systemic necrotizing vasculitides; however, such involvement has not been systematically assessed using cardiovascular magnetic resonance imaging (MRI).

Methods

Magnetic resonance angiography and contrast‐enhanced MRI were applied for the assessment of coronary arteries (the left anterior descending [LAD], left circumflex [LCx], and right coronary artery [RCA]) and myocardium, respectively, in 39 patients with vasculitis who were asymptomatic for cardiac disease (16 with microscopic polyangiitis [MPA], 11 with Wegener's granulomatosis [WG], 9 with Churg‐Strauss syndrome [CSS], and 3 with polyarteritis nodosa [PAN]). Data were compared with age‐matched disease‐control patients with rheumatoid arthritis (n = 20) or systemic lupus erythematosus (n = 13), and with healthy control individuals with normal coronaries (n = 40).

Results

Patients with MPA, WG, and PAN (but not with CSS) were found to display significantly increased maximal diameters of coronary arteries compared with healthy controls (for MPA and WG; P < 0.001 for LAD and RCA, and P < 0.01 for LCx) and with both disease‐control groups (for only MPA; P < 0.01 for LAD and RCA, and P < 0.05 for LCx). Fusiform coronary aneurysms were detected in patients with MPA (4/16) and PAN (2/3), whereas coronary ectasias were evident in patients with MPA (14/16) and WG (2/11). The presence of myocardial necrosis (by assessment of late gadolinium‐enhanced images) was identified only in patients with MPA (2/16) and CSS (3/8 studied).

Conclusion

Cardiovascular MRI assessment of patients with systemic vasculitis revealed coronary ectatic disease in the majority of patients with MPA and PAN, as well as in several patients with WG. Myocardial necrosis can be detected in MPA and CSS.     

Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients

OBJECTIVE: To determine the long-term outcome of patients with polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), to compare the long-term outcome with the overall French population, to evaluate the impact on outcome of the type of vasculitis, prognostic factors, and treatments administered at diagnosis, and to analyze treatment side effects and sequelae. METHODS: Data from PAN, MPA, and CSS patients (n = 278) who were enrolled between 1980 and 1993 were collected in 1996 and 1997 and analyzed. Two prognostic scoring systems, the Five-Factors Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS), were used to evaluate all patients at the time of diagnosis. RESULTS: The mean (+/- SD) followup of the entire population was 88.3 +/- 51.9 months (range 3 days to 192 months). Of the 85 deaths recorded, at least 41 were due to progressive vasculitis or its consequences. Death rates reflected disease severity, as assessed by the FFS (P = 0.004) and the BVAS (P < 0.0002), and the 2 scores were correlated (r = 0.69). Relapses, rarer in hepatitis B virus (HBV)-related PAN (7.9%) than in MPA (34.5%) (P = 0.004), occurred in 56 patients (20.1%) and did not reflect disease severity. Survival curves were similar for the subpopulation of 215 patients with CSS, MPA, and non-HBV-related PAN who were given first-line corticosteroids (CS) with or without cyclophosphamide (CYC). However, CS with CYC therapy significantly prolonged survival for patients with FFS scores > or =2 (P = 0.041). Relapse rates were similar regardless of the treatment regimen; only patients treated with CS alone had uncontrolled disease. CYC was associated with a greater frequency of side effects (P < 0.00001). CONCLUSION: Rates of mortality due to PAN (related or unrelated to HBV), MPA, and CSS reflected disease severity and were higher than the mortality rate in the general population (P < 0.0004). Rates of relapse, more common in MPA than HBV-related PAN patients, did not reflect disease severity. Survival rates were better among the more severely ill patients who had received first-line CYC. Based on these findings, we recommend that the intensity of the initial treatment be consistent with the severity of the disease. The use of the FFS and BVAS scores improved the ability to evaluate the therapeutic response.     

Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate

OBJECTIVE: To estimate the prevalences of polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), and Churg-Strauss syndrome (CSS). METHODS: Cases were collected in Seine-St. Denis County, a northeastern suburb of Paris, which has 1,093,515 adults (> or =15 years), 28% of whom are of non-European ancestry. The study period encompassed the entire calendar year 2000. Cases were identified by general practitioners, the departments of all the public hospitals and 2 large private clinics, and the National Health Insurance System. The Chapel Hill nomenclature was used to define MPA, and American College of Rheumatology criteria to define WG and CSS; PAN was diagnosed based on clinical laboratory, histological and/or angiographic findings. Three-source capture-recapture analysis was performed to correct for incomplete case ascertainment. RESULTS: A total of 75 cases were retained and capture-recapture analysis estimated that 23.8 cases had been missed by any 1 of the 3 sources. Accordingly, prevalences per 1,000,000 adults (95% confidence interval [95% CI]) were estimated to be 30.7 (95% CI 21-40) for PAN, 25.1 (95% CI 16-34) for MPA, 23.7 (95% CI 16-31) for WG, and 10.7 (95% CI 5-17) for CSS. The overall prevalence was 2.0 times higher for subjects of European ancestry than for non-Europeans (P = 0.01). CONCLUSIONS: This study provides the first prevalence estimates for these 4 vasculitides for a multiethnic, urban population. The significantly higher prevalence observed for Europeans may infer a genetic susceptibility of Caucasians. Compared with previous estimates based mostly on rural populations, the higher frequency of PAN and the lower frequency of WG might suggest specific environmental etiologic factors.     

Circulating cytokines and soluble CD23, CD26 and CD30 in ANCA-associated vasculitides

OBJECTIVE: To assess circulating immunoregulatory cytokines and soluble surface markers of T and B cell activation in the plasma of patients with Wegener's granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangiitis (MPA) during active and inactive disease, in order to establish their value in discriminating between disease entities and as markers of disease activity. METHODS: Plasma levels of IL-4, IL-5, IL-10, IL-12, IL-13, IFN-gamma and soluble CD23, CD26 and CD30 were determined by enzyme-linked immunosorbent assay in patients with WG (n = 21), CSS (n = 19) and MPA (n = 14) during active disease and remission. RESULTS: Concerning cytokines, no differences were observed for IFN-gamma, IL-4, IL-5 and IL-13. Plasma levels of IL-12 were decreased in all subgroups of patients. On the contrary, IL-10 levels were significantly elevated only in patients with CSS. Levels of sCD30 were significantly increased in patients with active generalized WG and CSS, but not in those with MPA and localized WG, correlating with the disease extent and activity. sCD26 levels were markedly decreased in patients with generalized WG, CSS and MPA and increased towards remission. sCD23 levels were slightly, but not significantly increased in CSS and generalized WG. CONCLUSION: Regarding the investigated immunoregulatory cytokines (Th1/Th2 type), only the measurement of plasma levels of IL-10 discriminated CSS from WG and MPA. The reported data could indicate a similar status of T cell activation in generalized WG and CSS, and possibly a shift in peripheral immunity towards a more humoral dominated immune response. The differences observed between patients with the localized and generalized forms of WG seem to reflect the clinically known biphasic course of this disease.     

Malignancy is increased in ANCA-associated vasculitis

OBJECTIVE: In the light of previous reports of an association between malignancy and renal vasculitis, we aimed to investigate the association of malignancy in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, either Wegener's granulomatosis (WG) or microscopic polyangiitis (MPA), and compare it with the general population and disease control groups comprising patients with systemic lupus erythematosus (SLE) or Henoch-Schonlein purpura (HSP). METHODS: A retrospective review of 200 consecutive patients with WG or MPA was performed. Malignancies preceding or concurrent with vasculitis were recorded and the incidence of malignancy was compared with those in a population of 129 patients with HSP, 333 patients with SLE and a normal population in the West Midlands of the UK. RESULTS: Twenty patients had a diagnosis of malignancy, 14 had MPA and six had WG. Patients with ANCA-associated vasculitis had an increased risk of malignancy compared with HSP patients, of whom six patients had malignancy (relative risk 0.85, confidence interval 0.69-1.05; P = 0.034), or SLE patients, of whom five patients had malignancy (relative risk 0.31, 95% confidence interval 0.14-0.7; P<0.0001). The rate of malignancy compared with an age-matched control group was increased in patients with ANCA-associated vasculitis and HSP (ANCA-associated vasculitis, relative risk 6.02, 95% confidence interval 3.72-9.74; HSP, relative risk 5.25, 95% confidence interval 2.4-11.5). The presence of ANCA was not predictive of malignancy. CONCLUSION: In conclusion, patients with ANCA-associated vasculitis have an increased risk of preceding or concurrent malignancy.     

重组人内皮细胞抗原成分α烯醇化酶的表达及其在弥漫性结缔组织疾病中的意义

目的 对蛋白质组学分离和鉴定的抗内皮细胞抗体(AECA)相关抗原-α烯醇化酶进行基因重组和表达,榆测其特异性抗体在血管炎和其他自身免疫性疾病患者血清中的阳性率.方法 聚合酶链反应(PCR)扩增人enol基冈CDS片段并与表达载体重组,在大肠杆菌中表达和纯化重组人α烯醇化酶.用质谱鉴定后的重组蛋白作为抗原,用点印迹法和酶联免疫吸附试验(ELISA)法分别检测健康人和不同结缔组织病患者血清中抗人α烯醇化酶抗体水平.结果 克隆了人enol基因cDNA片段.将重组的人α烯醇化酶在大肠杆菌中进行原核表达和纯化.用重组蛋白作为抗原,点印迹法检测健康对照和不同结缔组织病患者血清中抗人α烯醇化酶抗体,证实:①系统性血管炎(SV)、系统性红斑狼疮(SLE)、干燥综合征(SS)和类风湿关节炎(RA)患者血清中抗α烯醇化酶抗体的阳性率分别为76.7%、78.3%、63.6%和78.9%,均明显高于多发性肌炎/皮肌炎(PM/DM)组和健康对照组(P<0.01).②系统性血管炎中自塞病(BD)、多发性大动脉炎(TA)、韦格纳肉芽肿病(WG)、显微镜下多动脉炎(MPA)和Churg-Strauss综合征(CSS)患者血清中抗α烯醇化酶抗体阳性率各为74.0%、81.5%、62.5%、92.3%和80.0%.③SV和SLE患者血清中抗α烯醇化酶抗体的阳性率与SS和RA患者血清中该抗体的阳性率差异无统计学意义(P>0.05).④不同患者血清中抗α烯醇化酶抗体强阳性者所占比例分别为:SV 51.7%,SLE 33.3%,SS42.9%,RA 20.0%.RA组与SV组差异有统计学意义(P<0.05).结论 SV和SLE、SS、RA患者血清中均存在不同水平的抗α烯醇化酶抗体,α烯醇化酶是AECA识别的自身抗体之一.     

Neutrophil CD64 expression in Behçet's disease

OBJECTIVE: Hyperfunction of neutrophils is a characteristic finding in Beh?et's disease (BD). Microbial agents have been proposed as causative agents in the disease flares. Fc gamma receptor 1 (CD64) is not normally expressed by neutrophils of healthy individuals, but is upregulated by these cells in response to microbial wall components and proinflammatory cytokines. The degree of polymorphonuclear leukocyte (PMN) CD64 expression is different in autoimmune diseases and systemic infectious diseases. We investigated PMN CD64 expression in patients with BD. METHODS: Thirty-seven patients with active BD (M/F: 18/19, mean age: 34.4 +/- 9.7 yrs), 35 patients with inactive BD (M/F: 11/24, mean age: 35.9 +/- 11.6 yrs), 27 patients with culture proven infections (M/F: 19/8, mean age: 54.4 +/- 15.2 yrs), 31 healthy controls (M/F: 14/17, mean age: 37.7 +/- 8.7 yrs), and 42 patients with active inflammatory disease (M/F: 13/29, mean age: 39.3 +/- 14.9 yrs) were enrolled in this study. Flow cytometry was used to assess the prevalence of CD64-bearing PMN in whole blood samples. RESULTS: The prevalence of CD64-bearing PMN was significantly higher in patients with infectious disease (77.1 +/- 18.4), inflammatory disease (37.1 +/- 27.5), and active BD (48.9 +/- 22.5) than in healthy controls (9.5 +/- 7.8) or patients with inactive BD (12.9 +/- 9.5). CD64 expression was similar in controls and patients with inactive BD. In the infectious disease group, expression of CD64 was significantly higher than in the active BD and active inflammatory disease groups, while there was no significant difference between the groups of patients with active BD and inflammatory disorders. CONCLUSION: Neutrophil CD64 expression increases during exacerbation of BD. This increase appears to be a non-specific inflammatory response and does not reflect PMN activation triggered by a living microorganism.     

Prevalences of polyarteritis nodosa,microscopic polyangiitis,Wegener's granulomatosis,and Churg‐Strauss syndrome in a French urban multiethnic population in 2000: A capture–recapture estimate

Objective

To estimate the prevalences of polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), and Churg‐Strauss syndrome (CSS).

Methods

Cases were collected in Seine–St. Denis County, a northeastern suburb of Paris, which has 1,093,515 adults (≥15 years), 28% of whom are of non‐European ancestry. The study period encompassed the entire calendar year 2000. Cases were identified by general practitioners, the departments of all the public hospitals and 2 large private clinics, and the National Health Insurance System. The Chapel Hill nomenclature was used to define MPA, and American College of Rheumatology criteria to define WG and CSS; PAN was diagnosed based on clinical laboratory, histological and/or angiographic findings. Three‐source capture–recapture analysis was performed to correct for incomplete case ascertainment.

Results

A total of 75 cases were retained and capture–recapture analysis estimated that 23.8 cases had been missed by any 1 of the 3 sources. Accordingly, prevalences per 1,000,000 adults (95% confidence interval [95% CI]) were estimated to be 30.7 (95% CI 21–40) for PAN, 25.1 (95% CI 16–34) for MPA, 23.7 (95% CI 16–31) for WG, and 10.7 (95% CI 5–17) for CSS. The overall prevalence was 2.0 times higher for subjects of European ancestry than for non‐Europeans (P = 0.01).

Conclusions

This study provides the first prevalence estimates for these 4 vasculitides for a multiethnic, urban population. The significantly higher prevalence observed for Europeans may infer a genetic susceptibility of Caucasians. Compared with previous estimates based mostly on rural populations, the higher frequency of PAN and the lower frequency of WG might suggest specific environmental etiologic factors.

     

Evaluation of the Sørensen diagnostic criteria in the classification of systemic vasculitis

OBJECTIVES: To evaluate the use of the diagnostic criteria for Wegener's granulomatosis (WG) and microscopic polyangiitis (mPA) proposed by S?rensen et al. in the classification of primary systemic vasculitis (PSV). METHODS: We applied to our cohort of PSV patients the American College of Rheumatology (ACR) criteria for WG, Churg-Strauss syndrome (CSS) and polyarteritis nodosa (PAN), the Chapel Hill Consensus Conference (CHCC) definitions for WG, mPA and CSS, the Hammersmith criteria for CSS and the S?rensen criteria for WG and mPA. RESULTS: Ninety-nine PSV cases were identified. Fifty-six fulfilled criteria for WG (ACR), 60 for PAN (ACR) and 15 for CSS (ACR). Four fulfilled the Hammersmith criteria for CSS. Thirty-nine were defined as mPA (CHCC). Fifty-three patients fulfilled the S?rensen criteria for WG and three for mPA. Five of six patients classified as WG (ACR) who did not meet the S?rensen criteria were excluded by eosinophilia. Six patients who did not fulfil WG (ACR) met the S?rensen criteria for WG. CONCLUSION: The classification of systemic vasculitis is complicated and many cases fulfil more than one set of criteria. The S?rensen criteria for WG is limited by its exclusion of eosinophilia despite reports of an association. We recommend that tissue eosinophilia or peripheral eosinophilia of <1.5x10(9)/l should not exclude a diagnosis of WG. With this modification, the S?rensen criteria for WG may be a useful method of classification, especially in confirming the classification of WG in patients who fulfil both WG (ACR) and mPA (CHCC). Few patients fulfilled the S?rensen criteria for mPA which suggests that they are not of value in classification.