抗中性粒细胞胞质抗体相关性小血管炎伴肾损害26例临床分析

目的 分析抗中性粒细胞胞质抗体(ANCA)相关性小血管炎伴肾损害的临床特点及疗效.方法 对26例ANCA相关性小血管炎伴肾损害患者的临床表现、实验室检查指标、肾脏穿刺病理、治疗方案及转归等资料进行回顾性分析.结果 26例临床表现为呼吸道症状、消化道症状、肾损害及肾衰竭.实验室检查可见红细胞沉降率增快、C反应蛋白增高、血白细胞增高、血红蛋白降低和低蛋白血症.15例肾脏穿刺病理都符合小血管炎改变伴球性硬化及新月体形成,其中8例表现寡或无免疫复合物沉积,5例表现有颗粒状沉积.结论 ANCA相关性小血管炎伴肾损害临床表现具有复杂性、多样性,但随着ANCA监测技术的成熟和推广,使得早期诊断ANCA相关性小血管炎伴肾损害成为可能,有效改善了患者的预后.     

肺原发性小血管炎21例分析

目的探讨肺小血管炎的疾病特点及诊断,为血管炎的临床诊断提供借鉴。方法回顾性分析我院2002年1月至2010年12月21例肺小血管炎患者临床资料,其中12例为ANCA阳性患者,2例为病理确诊患者,余7例为临床诊断患者。结果肺小血管炎的临床表现无特异性;ANCA及病理学检查有助于早期诊断。结论原发性肺小血管炎影像表现无明显特异性,应综合分析提高对该病的认识。     

25例ANCA相关性肾小球肾炎临床观察

目的：探讨ANCA相关性肾小球肾炎的临床特点、治疗，以提高诊治水平。方法回顾性分析25例ANCA相关性肾小球肾炎的诊断和治疗。结果 ANCA相关性肾小球肾炎诊断中以蛋白尿、贫血为最常见，其次为消化道症状，再次为发热、咯血等症状。预后不良症状主要为咯血、急性肾衰竭。结论重视ANCA相关性肾小球肾炎的诊断，积极治疗，加强重症ANCA相关性肾小球肾炎患者的处置，对改善患者预后有关。     

肺脏受累的原发性小血管炎5例临床分析

目的提高对肺脏爱累的原发性小血管炎的认识，减少误诊率。方法回顾性分析5例肺脏受累的原发性小血管炎的病例资料。结果肺脏受累的原发性小血管炎患者临床表现为咳嗽、咳痰、痰血、发热、气喘、血尿、皮疹等。胸部X线或胸部CT表现为两肺浸润影或结节影。病理表现为血管壁炎症和坏死。早期易误诊为肺部感染、结核、肿瘤、间质性肺炎等。应用糖皮质激素和环磷酰胺治疗，肺部病变可明显吸收。结论伴肺脏受累的原发性小血管炎临床表现复杂多样，实验室检查无特异性，诊断困难，误诊率高，易延误病情，须引起临床医师的重视。     

抗中性粒细胞胞浆抗体相关性血管炎的临床特点与治疗

[目的]分析抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的临床表现及治疗.[方法]对我院2005年4月～2010年4月收治的47例AAV患者的临床资料进行回顾性分析.[结果]本组47例患者中,38例(80.9%,38/47)环核型ANCA(pANCA)阳性,均识别髓过氧化物酶(MPO),8例(17.0%,8/47)胞浆型ANCA(cANCA)阳性,均识别蛋白酶3(PR3),1例cANCA及pANCA均阳性,同时识别MPO及PR3.6个月内确诊者27例(57.4%,27/47),6个月以上确诊者20例(42.6%,20/47).临床表现呈多器官受累,其中肾脏受累43例(91.5%,43/47),血肌酐升高36例(76.6%,35/47),肺脏受累30例(63.8%,30/47),咯血16例(34.0%,16/47).此外还有不同程度的消化系统、关节、肌肉、耳、眼、皮肤、神经系统、鼻等器官损害及非特异性表现.80%上患者有血沉增快、C反应蛋白升高及贫血.主要采用糖皮质激素联合环磷酰胺治疗,诱导缓解期总缓解率75.9%.[结论]抗中性粒细胞胞浆抗体(ANCA)相关性血管炎临床表现为多器官受累,ANCA检测有助于早期诊断.     

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.     

原发性小血管炎的早期诊断和治疗

原发性小血管炎(primary small vessel vasculitis,PSV)是一组主要累及小血管(毛细血管、小静脉、微小动脉等),以血管壁坏死性炎性反应、纤维素样坏死为病理特征的一类自身免疫性疾病,包括显微镜下多血管炎(microscopic polyangiitis,MPA)、韦格纳肉芽肿(Wegener granulomatosis,WG)、过敏性肉芽肿性血管炎(Churg-Strauss syndrome,CSS)、抗中性粒细胞胞质抗体(ANCA)相关性坏死性新月体性肾炎(necrotizing crescentic glomerulonephritis,NCGN)[1],因其相当部分与ANCA关系密切,因而也称为ANCA相关性小血管炎(ANCA associated systemic vasculitis,AASV).     

甲亢合并抗肾小球基底膜抗体伴抗中性粒细胞胞浆抗体阳性肾炎1例

抗中性粒细胞胞浆抗体（ANCA） 是原发性小血管炎重要的血清学诊断指标,抗肾小球基底膜抗体（Anti-GBM） 是Anti-GBM 相关肾炎重要的血清学标志物.随着对Anti-GBM及ANCA 检测方法学的进展,一部分患者血清中可同时检测到Anti-GBM 和ANCA,并形成一种独立的临床病理类型.但甲亢合并Anti-GBM 和（ 或）ANCA 阳性肾炎患者的病例国内外少有报道,本文报道1 例,并分析其临床特点.     

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。     

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...     

ANCA-associated necrotizing/granulomatous glomerulonephritis in an elderly patient; importance of the renal biopsy

ANCA-associated glomerulonephritis may present initially with renal symptoms, especially in elderly patients. We report a case of ANCA-associated glomerulonephritis in an 80-year-old female presenting with renal insufficiency and proteinuria. There was no evidence of systemic illness at admission. The major finding in the renal biopsy was the presence of granulomas. Additional testing for anti-neutrophil cytoplasmic antibodies (ANCA) was suggested. The ANCA test was positive confirming the diagnosis of ANCA-associated necrotizing/granulomatous glomerulonephritis. The patient responded well to adequate treatment with immunosuppressive therapy. This case demonstrates the importance of the renal biopsy in cases of ANCA-associated glomerulonephritis presenting with renal symptoms.     

误诊为肺部感染的抗中性粒细胞胞浆抗体相关性小血管炎的CT表现

目的探讨以肺部症状为首发的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎误诊为肺部感染的CT表现,旨在提高对该类疾病的肺部影像学认识,减少误诊率。方法回顾10例经穿刺活检及临床证实的以肺部症状为首发的ANCA相关性小血管炎误诊为肺部感染的CT表现,结合文献进行分析。结果在10例以肺部症状为首发的ANCA相关性小血管炎中,肺内弥漫性磨玻璃影两例,占20.0%,弥漫粟粒影1例,占10.0%;间质性改变3例,占30.0%,其中网格状2例,蜂窝状1例,小叶间隔增厚两例;多发斑片及片状影4例,占40.0%,边缘模糊,密度不均,其中充气支气管征3例、伴多发结节3例、形成空洞两例;伴有纵隔淋巴结肿大3例、胸腔积液3例、心包积液1例;误诊为普通间质性肺炎3例、细菌性肺炎3例、粟粒型肺结核、浸润型肺结核、卡氏肺孢子虫肺炎及真菌性肺炎各1例。结论以肺部症状为首发的ANCA相关性小血管炎肺部CT表现复杂多样,易误诊为感染性病变,应结合临床实验室及病理检查作出正确诊断。     

Membranoproliferative glomerulonephritis in patients with cryoglobulinemia complicating hepatitis C virus: report of 11 cases

BACKGROUND: Membranoproliferative glomerulonephritis in patients with cryoglobulinemia complicating hepatitis C virus have yet been reported. Although, it remains controversial, antiviral treatment seems to be able to improve the outcome of glomerulonephritis. AIM: The objectives of the study were to analyze characteristics of this association and to report literature data and newness treatment. METHODS: It's a retrospective study including 11 patients with membranoproliferative glomerulonephritis, hepatitis C virus and mixed cryoglobulinemia. Hepatitis C virus antibodies was identified by ELISA technique. Hepatitis C virus genotype was identified in one patient. Cryoglobulins were isolated from sera of all patients at 37 degrees Celsius. RESULTS: Patients were 3 men and 8 women with a mean age of 51.9 +/- 15.5 years. Between the 11 patients, 7 had hypertension, 9 had nephrotic syndrome and 10 had chronic renal failure. Renal biopsy showed membranoproliferative glomerulonephritis lesions in all cases with fibrinoid thrombi in 8 cases. Six patients had chronic liver disease. Liver biopsy was performed in 4 patients, showing histological feature compatible with chronic active hepatitis in 2 cases. No patient had antiviral therapy. Renal failure was stable in 5 cases and progressed in 6 cases with end stage renal failure in 3 of them. One patient died, 4 months after diagnosis, because of severe pulmonary involvement in cryoglobulinemic vasculitis. In literature, treatment is dominated by antiviral therapy composed first by Interferon Alpha alone. Combination therapy associating Interferon and Ribavirin was recently used in renal involvement; it is clearly more effective than interferon alpha alone. CONCLUSION: Hepatitis C virus detection should be performed when membranoproliferative glomerulonephritis is associated with cryoglobulinemia. Antiviral treatment should be more widely used in Tunisia to evaluate his effect on renal involvement     

Silica exposure and systemic vasculitis

Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis.     

老年肾病综合征120例临床和病理特点

目的探讨、分析老年肾病综合征（NS）的临床与肾病理特点。方法回顾性分析2008年1月~2010年12月某院〉60岁120例老年NS患者的临床表现和肾活检病理诊断类型及其之间的关系。结果男性75例,女性45例,平均年龄（69.9±5.1）岁。120例NS患者中合并肾炎综合征有56例（46.7%）,合并的其他主要疾病有高血压（50例）、慢性肾病（34例）、2型糖尿病（23例）、冠心病（19例）、急性肾损伤（10例）。肾活检病理诊断中膜性肾病62例（51.7%）;其余分别为局灶节段性肾小球硬化症11例、系膜增殖性肾炎9例、IgA肾病8例、微小病变7例、糖尿病肾病5例、糖尿病肾病合并膜性肾病2例、淀粉样变肾病3例、紫癜性肾炎3例、肾小球轻微病变2例、ANCA相关性肾炎2例、多发性骨髓瘤肾损害、慢性间质性肾炎、狼疮性肾炎各1例。结论老年人发生NS临床表现与年轻患者显著不同,老年NS的病理改变仍以膜性肾病为主,存在多种继发因素。     

Clinical thinking and decision-making in practice. A patient with arthritis, skin lesions and a perforated nasal septum]

A man aged 38 years had arthritis, skin lesions and a perforation of the nasal septum. He had slight deterioration of renal function and a few granular casts in the urine. Antineutrophil cytoplasmic antibody and other immune parameters were normal. A biopsy of the nasal septum did not show granulomatous lesions. The differential diagnosis on the available clinical grounds could be narrowed to Wegener's disease, Henoch-Sch?nlein vasculitis or microscopic polyangiitis. A biopsy of the skin showed leukocytoclastic vasculitis, with IgA and IgM depositions compatible with Henoch-Sch?nlein vasculitis. A renal biopsy showed small segmental necrotising lesions of the glomerular capillaries, with minimal extracapillary proliferation; immune fluorescence did not detect any significant depositions. The picture was compatible with Wegener's disease. A nasal septum defect together with glomerulonephritis, which could be suspected from only minimal abnormalities in the urine, is almost conclusive for the diagnosis.     

Gastrointestinal surgery and gastroenterology. XIV. Mesenteric abnormalities in generalised vascular disease

Various forms of vasculitis may result in mesenteric ischaemia, ischaemic colitis or aneurysm formation in the aorta or intestinal blood vessels. Vasculitides may involve large- and/or medium-sized vessels, medium- and/or small-sized vessels, or small-sized vessels only. It is essential to differentiate between the different forms of vasculitis since diagnostic tests and therapies differ greatly. Gastrointestinal manifestations of vasculitis can generally be detected using angiography, digital subtraction angiography and/or magnetic resonance angiography (MRA). Various laboratory tests are helpful in establishing the diagnosis in patients in whom vasculitis is clinically suspected. In addition, the diagnosis should be confirmed using histology or angiography if possible. Treatment of vasculitis not caused by chronic infection consists of high dose corticosteroids and, in the case of polyarteritis nodosa or vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA), cyclophosphamide.     

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。     

Delayed ANCA positivity in pulmonary-renal syndrome

A 57-year-old man was admitted due to rapidly progressive renal failure and pulmonary edema. Chest X-ray showed a bilateral lung infiltrate, while a normal myocardial contractility was reported by echocardiography. Though initially ANCA were absent, a necrotizing vasculitis with polymorphonuclear leukocyte infiltrate was observed in a kidney biopsy specimen. Renal histology was compatible with microscopic polyangiitis because of necrotizing lesions located at small vessels and at glomeruli that were not crescentic. Corticosteroids and immunosuppressive treatment was played with significant clinical improvement. Six months later, the patient died of a gastrointestinal hemorrhage due to bowel perforation by vasculitic lesion. This time high p-ANCA positivity was detected and at renal histology crescentic glomeruli were observed.