抗中性粒细胞胞浆抗体相关性血管炎的肾脏损害和病理(附16例分析)

[目的]观察抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的肾脏损害和病理表现.初步探讨ANCA相关性血管炎的治疗方法及预后.[方法]回顾性分析2005～2009年某院确诊的ANCA相关性血管炎患者的临床资料及肾脏病理.[结果]16例ANCA相关性血管炎患者中,微型多血管炎(MPA)12例,韦格纳肉芽肿(WG)4例.环核型ANCA(pANCA)阳性12例,均识别髓过氧化物酶(MPO);胞浆型ANCA(cANCA)阳性5例,均识别蛋白酶3(PR3).全部研究病例均表现为多系统损害,肾脏为最常见累及器官(累及率为100%),镜下血尿15例(93.8%),血肌酐升高15例(93.8%),16例血肌酐平均489.2μmol/L(105～1096μmol/L).急进性肾小球肾炎8例(50%),急性肾功能衰竭3例(18.8%),慢性肾功能衰竭4例(25.0%).肾脏病理:寡免疫复合物沉积性新月体肾炎10例(62.5%),伴肾小球毛细血管袢纤维素样坏死7例(43.8%),肾小管间质不同程度炎性细胞漫润9例(56.3%).经激素联合环磷酰胺或激素联合吗替麦考酚酯、血液透析等治疗.其缓解率为56.3%.死亡率为18.8%.[结论]AAV患者肾损害严重,寡免疫复合物沉积性新月体性肾小球肾炎、伴肾小球毛细血管袢纤维素样坏死是其肾脏病理的主要特点;ANCA联合检测有助于诊断;肾功能损害重及合并感染者,预后差.     

52例抗中性粒细胞胞浆抗体相关性血管炎患者临床表现及实验室检查分析

目的探讨抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(ANCA associated vasculitis,AAV)临床表现及实验室检查指标,为临床诊断提供依据。方法选取2013年7月-2016年4月在湖南省人民医院住院的52例MPO-AAV患者为ANCA阳性相关性血管炎组(AAV组),对照组为同期在本院住院的48例非AAV自身免疫病患者。回顾性分析2组患者的性别、年龄、科室分布、实验室检查、临床表现等特征。结果 AAV组就诊于风湿免疫科居首位(44.23%),其次为呼吸科(15.38%)。对照组患者首次就诊于风湿免疫科居首位(52.08%),其次为神内二科(18.75%)。与对照组相比,AAV组年龄、发热、呼吸系统和肾脏受累差异有统计学意义(P0.05);AAV组MPO、ESR、hs-CRP明显高于对照组(P0.05),HGB明显低于对照组(P0.05)。Logistic多因素结果显示,MPO和hs-CRP与AAV相关(P0.05)。结论 AAV患者以中老年人为主,肾脏和肺受累多见,MPO和hs-CRP对AAV诊断有指导意义。     

抗内皮细胞抗体与抗中性粒细胞胞浆抗体的临床应用价值

[目的]探讨抗内皮细胞抗体(AECA)、抗中性粒细胞胞浆抗体(ANCA)在自身免疫性疾病及血管炎中的相关性及临床应用价值.[方法]168例自身免疫性疾病及血管炎患者血清的AECA、c-ANCA、p-ANCA应用间接免疫荧光分析法(IIF)检测,MPO-ANCA、PR3-ANCA应用酶联免疫吸附法(ELISA),分析各组病人血清AECA及ANCA阳性率及滴度,AECA与ANCA的相关性.[结果]RA组AECA阳性率27.73%(33/119);ANCA阳性率20.17%(24/119):AECA+ANCA合并阳性率42.86%(51/119).SLE组AECA阳性率8.00%(2/25),而ANCA阳性率40.00%(10/25):AECA+ANCA台并阳性率44.00%(11/25).肾脏疾病组AECA阳性率41.67%(10/24).[结论]AECA、ANCA在风湿病血管损害的辅助诊断中具有良好的应用前景;AECA与ANCA二者之间无相关性,联合检测AECA和ANCA将可以提高它们在临床应用上的敏感性.     

显微镜下多血管炎1例并文献复习

目的　对显微镜下多血管炎 (MPA)患者诊断、治疗进行研究。方法　收集和分析 1例MPA的临床、实验室、血清免疫学及影像学特点并复习相关资料。结果　MPA与免疫因素有关 ,是一种累及小血管的系统性坏死性血管炎 ,可侵犯肾、肺、关节等多脏器 ,肺受损是MPA早期主要表现之一。抗中性粒细胞胞浆抗体 (ANCA)的监测为诊断部分原发性小血管炎的血清学工具 ,髓过氧化物酶 (MPO)为pANCA的主要靶抗原 ,是确诊及判断疾病活动的重要指标之一。结论　有典型的肺 -肾综合征 ,加之 pANCA (+) (MPO10 0 % ) ,即使没有肾活检也可以确定MPA ,确诊后 ,尽早应用甲基强的松龙及环磷酰胺冲击 ,预后良好     

ANCA相关性小血管炎并肾脏损害的临床分析

目的探讨抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎并肾炎的临床诊治方法。方法对20例确诊为ANCA相关性小血管炎并肾炎的患者临床资料进行总结分析。结果ANCA相关性小血管炎并肾炎临床表现多样,全身各系统均可受累,以肺、肾受累为主。一旦肾脏受累,表现为RPGN,经皮质激素,细胞毒药物治疗,部分忠者肾功能得到改善,脱离透析治疗。结论ANCA相关性小血管炎并肾炎临床表现多样,早期诊断,早期适当治疗可改善患者预后。     

首发于中枢神经系统的急性显微镜下多血管炎一例及报告

显微镜下多血管炎( microscopic polyangiitis,MPA)与肉芽肿性多血管炎和嗜酸性肉芽肿性多血管炎一起构成抗中性粒细胞胞浆抗体( antineutrophil cytoplasmic antibodies, ANCA)相关性血管炎[1].研究发现[2] ,MPA多见于欧美国家,临床表现复杂多样、无...     

25例ANCA相关性血管炎肾损害患者的护理

抗中性粒细胞胞浆抗体(ant i neutrophil cytoplasmic ant ibodies,ANCA)相关性血管炎是一类起病隐匿的自身免疫性疾病,可累及多个器官和系统。其中,肾累及率高达80%以上,是ANCA相关性血管炎损害的最主要靶器官之一,且多进展迅速,严重者可危及患者生命[1]。早期诊断、及时合理的     

抗中性粒细胞胞浆抗体在炎症性肠病中的诊断意义

目的 研究炎症性肠病患者抗中性粒细胞胞浆抗体(ANCA)的阳性率、特异性及与炎症程度病变范围的相关性,探讨ANCA作为溃汤性结肠炎患者辅助诊断指标的价值。方法 应用间接免疫荧光法检测42例溃疡性结肠炎患者、32例非溃疡性结肠炎引起的腹泻患者及35例正常对照者血清ANCA阳性率。结果 溃疡性结肠炎组pANCA阳性率为69.1％。非溃疡性结肠炎组总的pANCA阳性率为3.5％,P<0.05。pANCA对溃疡性结肠炎诊断的特异性为93.8％。核周型抗中性粒细胞胞浆抗体(pANCA)阳性率与病变范围无关(P>0.05)。结论 ANCA对炎症性肠病特别是溃疡性结肠炎的辅助诊断具有意义,同时具有较高的鉴别诊断价值。     

FcγRⅡa及相关炎症分子在ANCA相关性血管炎中的意义

目的探讨中性粒细胞膜上FcγRIIa表达与ANCA相关性血管炎(AAV)活动性的关系及相关细胞因子的变化。方法收集61例AAV患者(其中活动组33例,静止组28例),60例健康者作为对照,采用流式细胞术(FACS)分析血液中性粒细胞膜上FcγRIIa表达,并应用酶联免疫吸附试验(ELISA)检测血清细胞因子TNF-α、IL-6及IL-8的水平。结果 AAV活动组血液中性粒细胞中表达FcγRⅡa的比例较静止组及对照组高(P0.01);AAV活动组血清TNF-α、IL-6及IL-8的水平也明显高于静止组及健康对照组(P0.01)。结论中性粒细胞膜上FcγRIIa及血清细胞因子TNF-α、IL-6及IL-8的水平可作为评价AAV活动性的分子指标,ANCA可能通过FcγRIIa影响细胞因子TNF-α、IL-6及IL-8的水平引起AAV。     

抗中性粒细胞胞浆抗体在原发性小血管炎肾脏损害中的作用

原发性小血管炎及其肾损害是近几年的研究热点之一。1994年美国Chapel Hill系统性血管炎分类命名意见按受累血管的大小将系统性血管炎分为大血管炎、中血管炎和小血管炎[1]。原发性小血管炎包括韦格纳肉芽肿病( Wegener granulo-matosis ,WG)、显微镜下型多血管炎( microscopic polyangiitis ,MPA)、变应性肉芽肿血管炎(Churg -Strauss syndrone ,CSS)、过敏性紫癜、原发性冷球蛋白血症血管炎、皮肤白细胞破碎性血管炎。这6种原发性小血管炎都可发生肾脏病变,但并非都有抗中性粒细胞胞浆抗体(ANCA)阳性,其中WG、MPA、CSS为AN…     

Survey of ethylenethiourea (ETU) in ethylenebis(dithiocarbamate) (EBDC) fungicides

Ethylenethiourea (ETU) has been recognized as a compound with adverse toxicological properties. It may occur in ethylenebis(dithiocarbamate) (EBDC) fungicides as a by-product and/or a degradation product. Restrictions have been issued by the Italian Health Authority to keep the level of ETU in EBDC formulations under 0.5% (on an EBDC technical product basis). A survey was carried out on 85 samples of commercial formulations from the Italian market to verify compliance with the above limitation. ETU was extracted by methanol and determined by gas chromatography with a flame ionization detector. Three of the 85 samples (3.5%) exceeded the limit of 0.5% established by the Italian legislation for ETU.     

Diagnostic image (5) (Ileum pathology)

In a 28-year-old woman Crohn's disease was diagnosed. She had drug treatment but the situation deteriorated and ileocoecal resection was carried out. In the surgical specimen the diagnosis was confirmed.     

Crusted (Norwegian) scabies in Dakar (Senegal)

Between January 1993 and June 1997, 30 patients with crusted scabies were seen at the dermatological clinic in Dakar (Senegal). Seventeen of these patients were male and 13 were female. Six were children aged 5 to 15 years and 24 were adults aged 18 to 70 years. Diagnosis of crusted scabies was straightforward because this condition, which was rare in Dakar before 1990, has become much more common and is now familiar to Senegalese dermatologists. The infection presented as an extensive scaly or crusted eruption with symmetrical lesions affecting the hands, feet, knees, elbows and ears in particular. Scalp involvement was reported in 25 patients. Erythrodermia was present in 4 cases and pachyonychia in 4 cases. Twenty-seven of the 30 patients had moderate or severe pruritus, whereas an absence of pruritus is regarded as a classical characteristic of crusted scabies. Diagnosis was readily confirmed by examination of hyperkeratotic material under the microscope: numerous mites and eggs were present. The two most common etiological factors were auto-immune diseases (6 cases, 4 of whom were receiving no steroid or other immunosuppressive treatment at the time of onset of crusted scabies) and malnutrition (5 of the 6 children in the study). The other associated conditions identified were: physical debilitation (4 cases), HIV infection (3), mental disability-Down's syndrome (3) and long term use of topical steroids for artificial depigmentation (2). Two patients were immunocompetent and 5 patients died shortly after diagnosis, before any underlying conditions could be identified. Seven patients were cured with benzyl benzoate. Seven others, all adults, received a single oral dose of ivermectin (200 mg/kg) and topical kerolytic drugs. Ivermectin was ineffective in 1 case, and an improvement was observed in another case, although a complete cure was achieved only after a second dose. The other patients were all cured and showed no signs of scabies one month after ivermectin treatment. A recurrence was observed in 3 patients a few months later however, suggesting that these patients were reinfected. No side effects were reported in any of the patients treated with ivermectin.