ANCA相关性血管炎肾损害临床特征及早期诊治探讨

目的 分析抗中性粒细胞胞浆抗体(ANCA)相关性血管炎的临床表现和肾脏病理特征,探讨早期诊断和治疗对预后的影响.方法选取本院2000年1月至2009年8月明确诊断的ANCA相关性血管炎共21例,18例行肾活检.总结患者的临床病理资科.分析不同治疗时机对肾功能转归的影响.结果本组21例ANCA相关性血管炎平均年龄(52.5±11.5)岁,显微镜下多血管炎(MPA)16例,韦格纳肉芽肿(WG)3例,变应性肉芽肿性血管炎(CSS)2例.肾外表现主要为发烧17例(80.1%)、下呼吸道症状18例(85.7%)、肺影像学改变21例(100%)、贫血16例(76.2%)、眼耳鼻受累8例(38.1%);肾脏表现血尿21例(100%),蛋白尿19例(90.1%),血肌酐正常6例(28.5%),升高15例(71.4%),8例需透析替代.ANCA检测pANCA和MPO-ANCA阳性16例,cANCA和PR3-ANCA阳性3例.pANCA/MPO-ANCA和cANCA/PR3-ANCA均阳性1例,全阴性1例.肾活检可见节段性小血管壁纤维素样坏死,新月体多见.免疫荧光无或微量免疫复合物沉积.治疗采用糖皮质激素联合环磷酰胺,重症加用血浆置换.7例血肌酐异常但不需透析者5例治疗后血肌酐恢复正常;8例需透析者2例治疗后血肌酐恢复正常,2例脱离透析但血肌酐异常,4例未能脱离透析.结论ANCA相关性小血管炎临床表现多样,肺、肾是最常见的受累器官.ANCA检测和肾活检有助于早期诊断,尽早积极治疗有助于肾功能的恢复.     

原发ANCA相关性小血管炎的分子遗传学研究进展

患者血清中ANCA的出现和人类白细胞抗原系统参与的特征已反映出其自身免疫学发病的遗传学背景.因而当前倾向于认为原发性ANCA相关性小血管炎是在遗传易感性的基础上,由未知的环境因素和感染因素所诱发的自身免疫性疾病.本文从ANCA、T细胞及受体、细胞因子及HLA等方面入手,重点就近年来对该病分子遗传风险基因的研究进展作一综述并指出不足之处.为进一步探寻其遗传学发病病理机制提供一些线索.     

髓过氧化物酶抗中性粒细胞胞质抗体相关性肾小球肾炎患者的临床病理特征及肾脏预后分析

目的:研究髓过氧化物酶（myeloperoxidase,MPO）抗中性粒细胞胞质抗体（anti-neutrophil cytoplasmic antibody,ANCA）相关性肾小球肾炎（MPO-ANCA-glomerulonephritis,MPO-ANCA-GN）患者的临床病理特征以及患者肾脏预后的相关因素。方法:...     

抗中性粒细胞胞质抗体相关性血管炎的研究现状

抗中性粒细胞胞质抗体(ANCA)是存在于系统性血管炎患者中的一类自身抗体,其抗原是多形核粒细胞和单核细胞的胞质成分.除此之外,微型多动脉炎、局限性肾动脉炎等的发病与ANCA亦密切相关.临床上将上述疾病称为ANCA相关系统性血管炎(AASV).AASV肾脏损害的表现主要包括少尿、血尿和蛋白尿,伴肾小球滤过率下降.目前诊断主要依赖肾脏病理学检查,其特征性病理表现为寡免疫复合物沉积的局灶节段坏死性肾小球肾炎,形成以巨噬细胞和上皮细胞为主要细胞成分的新月体.AASV预后不良的因素包括老年、肾功能严重受损.肾脏病理为判断预后提供更多的依据,肾间质纤维化提示预后不良.     

系统性红斑狼疮并发继发性抗磷脂综合征肾损害11例临床病理分析

目的 分析系统性红斑狼疮（SLE）并发继发性抗磷脂综合征（APS）肾损害的临床病理表现,旨在提高对该类疾病的认识。 方法 回顾性分析北京协和医院2000年至2010年期间确诊SLE并发继发性APS（SLE伴APS）并行肾组织学检查的11例患者的资料,分析其临床病理特点,并比较其和SLE不伴APS患者在肾损害的临床病理及预后上的差异。 结果 11例SLE伴APS患者均有肾脏受累,突出表现为高血压（54.5%）、大量蛋白尿（≥3.5 g/d）（72.7%）和肾功能异常（45.5%）。SLE伴APS患者的舒张压、平均动脉压以及肾小球滤过率（eGFR）均明显高于SLE不伴APS患者（均P < 0.05）。8例（72.7%）SLE伴APS患者存在肾内血管的“血管闭塞性表现”,即符合抗磷脂综合征肾病（APSN）的病理表现,包括肾小血管、肾小球毛细血管血栓形成以及肾小动脉内膜增生、局灶性肾皮质萎缩、肾小管甲状腺样化,其中慢性APSN表现5例（45.5%）,急性APSN表现4例（36.4%）（其中1例同时有急性和慢性表现）;其APSN的发生率以及急性APSN的发生率明显高于SLE不伴APS患者（P < 0.05）。 结论 SLE并发APS肾损害患者除狼疮肾炎外,多并发APSN,临床上高血压和肾功能异常更为突出。     

狼疮性肾炎患者血浆与肾组织抗中性粒细胞胞浆抗体的表达

目的探讨抗中性粒细胞胞浆抗体（ANCA）在狼疮性肾炎（LN）患者血浆及肾组织中的表达及其意义。方法回顾性分析40例LN患者的临床资料及ANCA检测结果,按。肾脏病变活动性评分、系统性红斑狼疮疾病活动性指数（SLEDAI）评分、尿蛋白含量进行分类统计。结果血浆ANCA阳性率为37.5％,肾组织ANCA阳性率为42.5％,两者呈正相关性（r=0.765,P=0.013）,但无统计学差异。肾脏活动性病变组的血浆ANCA和肾组织ANCA阳性率均高于肾脏慢性化病变组（P〈0.05,P〈0.01）,高尿蛋白组肾组织ANCA阳性率明显高于低尿蛋白组（P〈0.01）。结论ANCA可作为判断狼疮性肾炎（LN）肾脏病变及临床活动性的重要参考指标。     

ANCA相关性血管炎的治疗进展

ANCA(抗中性粒细胞胞浆抗体,anti-neutrophil cytoplasm antibodies)相关性血管炎常包括韦格纳肉芽肿(Wegener's granulomatosis,WG)、显微镜下多血管炎(microscopic polyangiitis,MPA)及局限于肾脏的血管炎,以血循环中存在抗中性粒细胞胞浆抗体为主要特征,可导致快速进展型肾小球肾炎(RPGN),其共同的肾脏病理表现为新月体的形成.如不及时治疗,则能在短期内进展为终末期肾衰竭(ESRD),近年来针对ANCA相关性血管炎的治疗有较大进展,现综述如下.     

系统性硬化合并ANCA相关性小血管炎一例

我们收治了一例急性肾功能衰竭的系统性硬化（systemic sclerosis，SSc）患者，表现为非恶性高血压，ANCA阳性的新月体性肾炎，现报道如下。     

抗中性粒细胞胞质抗体相关性血管炎的研究现状

抗中性粒细胞胞质抗体(ANCA)是存在于系统性血管炎患者中的一类自身抗体,其抗原是多形核粒细胞和单核细胞的胞质成分.除此之外,微型多动脉炎、局限性肾动脉炎等的发病与ANCA亦密切相关.临床上将上述疾病称为ANCA相关系统性血管炎(AASV).AASV肾脏损害的表现主要包括少尿、血尿和蛋白尿,伴肾小球滤过率下降.目前诊断主要依赖肾脏病理学检查,其特征性病理表现为寡免疫复合物沉积的局灶节段坏死性肾小球肾炎,形成以巨噬细胞和上皮细胞为主要细胞成分的新月体.AASV预后不良的因素包括老年、肾功能严重受损.肾脏病理为判断预后提供更多的依据,肾间质纤维化提示预后不良.     

ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the "normotensive scleroderma renal crisis"

The scleroderma renal crisis is characterized by acute onset of severe hypertension and by rapidly progressive hyperreninemic renal failure. There is, however, a very limited subset of patients with rapidly progressive renal failure who remain normotensive and develop ANCA-positive crescentic glomerulonephritis. We report a case of normotensive acute renal failure secondary to anti-MPO antibody-associated crescentic glomerulonephritis in a patient with diffuse systemic sclerosis. She was referred to our department with normal blood pressure and no extrarenal clinical manifestation ofvasculitis. She presented with rapidly progressive renal failure, microscopic hematuria and minimal proteinuria. P-ANCA were positive by immunofluorescence, with ELISA-confirmed specificity for myeloperoxidase. Renal biopsy revealed typical features of pauciimmune glomerulonephritis with crescent formation and fibrinoid necrosis. The patient was initially treated with i.v. cyclophosphamide only. Because of ongoing deteriorating renal function, additional treatment with intravenous pulses of methylprednisolone followed by oral prednisone was started and allowed renal function improvement. After 9 months, serum creatinine had almost returned to normal level with minimal proteinuria, no hematuria and negative ANCA testing. Control kidney biopsy only revealed scar lesions. The association of ANCA-positive crescentic glomerulonephritis and systemic sclerosis is a very rare event. Treatment with intravenous cyclophosphamide and corticosteroids allows rapid and long-term improvement of renal function. The onset of typical scleroderma renal crisis triggered by high-dose corticosteroids is unlikely but requires a close follow-up of patients with overlapping systemic sclerosis. Diagnosis and treatment are discussed and previously published cases are reviewed.     

Acute kidney injury in children

Acute kidney injury (AKI) (previously called acute renal failure) is characterized by a reversible increase in the blood concentration of creatinine and nitrogenous waste products and by the inability of the kidney to regulate fluid and electrolyte homeostasis appropriately. The incidence of AKI in children appears to be increasing, and the etiology of AKI over the past decades has shifted from primary renal disease to multifactorial causes, particularly in hospitalized children. Genetic factors may predispose some children to AKI. Renal injury can be divided into pre-renal failure, intrinsic renal disease including vascular insults, and obstructive uropathies. The pathophysiology of hypoxia/ischemia-induced AKI is not well understood, but significant progress in elucidating the cellular, biochemical and molecular events has been made over the past several years. The history, physical examination, and laboratory studies, including urinalysis and radiographic studies, can establish the likely cause(s) of AKI. Many interventions such as ‘renal-dose dopamine’ and diuretic therapy have been shown not to alter the course of AKI. The prognosis of AKI is highly dependent on the underlying etiology of the AKI. Children who have suffered AKI from any cause are at risk for late development of kidney disease several years after the initial insult. Therapeutic interventions in AKI have been largely disappointing, likely due to the complex nature of the pathophysiology of AKI, the fact that the serum creatinine concentration is an insensitive measure of kidney function, and because of co-morbid factors in treated patients. Improved understanding of the pathophysiology of AKI, early biomarkers of AKI, and better classification of AKI are needed for the development of successful therapeutic strategies for the treatment of AKI.     

Normotensive scleroderma renal crisis: Case report and review of the literature

A 58 year old man presented with skin manifestations of scleroderma without systemic involvement. Within few weeks of oral corticosteroids and penicillamine therapy, rapidly progressive systemic sclerosis developed. The deterioration manifested by skin lesions, douse interstitial restrictive lung disease, acute renal failure with normal blood pressure values, and microangiopathic hemolytic anemia compatible with hemolytic uremic syndrome. Chronic renal failure developed and was treated by dialysis, but the patient died due to sepsis. The course of renal involvement was normotensive; antihypertensive therapy was not prescribed even once.

The association of scleroderma renal crisis with normal blood pressure is probably a rare and ominous combination.     

Scleroderma renal crisis with limited cutaneous sclerosis and positive anti-RNA polymerase antibody

A 59-year old Japanese woman diagnosed as having systemic sclerosis with limited cutaneous involvement was hospitalized with scleroderma renal crisis. Malignant hypertension with marked hyperreninemia and grade three hypertensive retinopathy were present, and the renal biopsy showed marked intimal thickening of the small intrarenal arteries. The patient's serum was negative for anticentromere and anti-DNA topoisomerase I (Scl-70) antibodies, whereas a radioimmunoprecipitation study revealed the presence of antibody to RNA polymerases I, II, and III. The patient was successfully treated with captopril, with normalization of blood pressure and reversal of the decreased renal function. The presence of antibody to RNA polymerases may be a marker for the development of life-threatening scleroderma renal crisis even in patients with limited cutaneous systemic sclerosis. Received: July 21, 1998 / Accepted: November 15, 1999     

Acute kidney injury and the critically ill

Acute renal failure is commonly encountered in the intensive care unit. It is associated with considerable morbidity and mortality. There are many possible aetiologies in the critically ill, including nephrotoxic agents, hypovolaemia and sepsis. While many classification systems for acute renal failure exist, the RIFLE (Risk, Injury, Failure, Loss, End-stage) criteria and the Acute Kidney Injury Network (AKIN) criteria are the most commonly utilized. Many supportive therapies are employed to minimize the degree of renal injury once recognized, such as fluid resuscitation and maintenance of an adequate mean arterial pressure (with the use of inotropes in persistent hypotension despite fluid and treatment of the underlying aetiology). However, if renal failure becomes established, then renal replacement therapy (RRT) may be needed to maintain homoeostasis. While there are no clear guidelines with respect to the ideal mode or timing of RRT, we will discuss pros and cons of the various bedside options.     

Acute kidney injury and the critically ill

Acute renal failure is commonly encountered in the intensive care unit. It is associated with considerable morbidity and mortality. There are many possible aetiologies in the critically ill, including nephrotoxic agents, hypovolaemia and sepsis. While many classification systems for acute renal failure exist, the RIFLE (Risk, Injury, Failure, Loss, End-stage) criteria and the Acute Kidney Injury Network (AKIN) criteria are the most commonly utilized. Many supportive therapies are employed to minimize the degree of renal injury once recognized, such as fluid resuscitation, maintenance of an adequate mean arterial pressure (with the use of vasopressors in persistent hypotension despite fluid and treatment of the underlying aetiology). However, if renal failure becomes established, then renal replacement therapy (RRT) may be needed to maintain homeostasis. While there are no clear guidelines with respect to the ideal mode or timing of RRT, we will discuss pros and cons of the various options.     

Acute kidney injury in peripheral arterial surgery patients: a cohort study

Abstract

Purpose: The aim of the study was to evaluate the prevalence of acute kidney injury (AKI) in a cohort of surgically treated patients with peripheral artery disease (PAD) and its association with the short-term and long-term outcome. Materials and methods: We conducted a retrospective cohort study on all the consecutive PAD inpatients in 2008. Data on the patients’ demographic characteristics, medical history, treatment, outcome and laboratory tests measurements were retrieved from the medical records. Results: We analyzed 166 patients (71.6% males, mean age 63.2 years?±?SD 10.7 years) and found an AKI prevalence of 12.7%. The AKI patients’ group had more chronic kidney disease (CKD) (23.8% vs. 6.2%, p?=?0.005), diabetes mellitus (DM) (61.9% vs. 33.1%, p?=?0.011) and a higher length of hospital stay (19.48 vs. 15.42 days, p?=?0.047). At one year, the mortality was 33.3% in the AKI group compared to 1.3% in non-AKI group, with a strong association between AKI and death (OR?=?35.7; 95%CI?=?6.7 to 189) and AKI and major cardiovascular events (OR?=?29.1; 95% CI?=?6.8 to 123.4). There was no significant difference in terms of age, cardiovascular disease and medication between the two groups. Conclusions: AKI was associated with a poorer one-year outcome after the surgery of PAD patients. In our study, the presence of previous chronic kidney disease and type 2 diabetes increased the incidence of acute kidney injury after surgery.     

Acute kidney injury in hematopoietic cell transplantation

Hematopoietic cell transplantation is becoming an increasingly common treatment modality for a variety of diseases. However, patient survival may be limited by substantial treatment-related toxicities, including acute kidney injury (AKI). AKI can develop in approximately 70% of patients posttransplant and is associated with an increased risk of morbidity and mortality. The development of AKI varies depending on the type of conditioning regimen used and the donor cells infused at the time of transplant, and the etiology often is multifactorial. Epidemiology, risk factors for development, pathogenesis, and potential treatment options for AKI in the hematopoietic cell transplantation population are reviewed as well as newer data on early markers of renal injury. As the indications for and number of transplants performed each year increases, nephrologists and oncologists will have to work together to identify patients who are at risk for AKI to both prevent its development and initiate therapy early to improve outcomes.     

从急性肾功能衰竭到急性肾损伤认识的转变谈麻醉管理对肾脏的保护

背景 对患者肾功能的保护是临床诊疗过程中的重要目标,改善全球肾病预后组织(kidney disease improvingglobal outcomes,KDIGO)曾先后多次制定了相关肾病的治疗指南. 目的 概述急性肾损伤(acute kidney injury,AKI)的相关研究及其临床意义. 内容 AKI概念的产生、围术期麻醉管理对肾功能的影响及AKI治疗的争议. 趋向 AKI体现了疾病动态演变的过程,提高了对肾损伤的认识,有利于围手术期肾功能的保护.