Dynamically changing antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis:A case report

BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.

Antineutrophil cytoplasmic antibodies crescentic allograft glomerulonephritis after sofosbuvir therapy

Antineutrophil cytoplasmic antibodies(ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis(RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine(adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection. Here, we report a case of ANCA vasculitis and RPGN following sofosbuvir administration in a kidney transplant recipient. It also represents the first case of drug-associated ANCA vasculitis in a transplanted kidney. Further drug monitoring is necessary to elucidate the degree of association and possible causal effect of sofosbuvir and perinuclear ANCA vasculitis.

Myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis with headache and kidney involvement at presentation and with arthralgia at relapse:A case report

BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and relapse manifestations have not been reported in myeloperoxidase(MPO)-AAV patients.CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache.Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria,serum creatinine of 243μmol/L,anti-MPO antibody titer of>400 RU/mL,and positive perinuclearantineutrophil cytoplasmic antibody.Renal biopsy showed pauci-immune crescentic glomerulonephritis.The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality.Therefore,MPO-AAV was diagnosed.Corticosteroids,plasmapheresis,and cyclophosphamide as induction therapy and mycophenolate mofetil(MMF)as maintenance therapy were administered.The patient’s headache disappeared;serum creatinine returned to normal;complete remission of microscopic hematuria and proteinuria was observed.Anti-MPO antibody titer reached normal limits after immunosuppressive treatment.Twenty-five months after stopping the immunosuppressive treatment,the patient relapsed with arthralgia,without neurological or renal involvement.The patient’s arthralgia improved after treatment with prednisone and MMF.CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement.However,relapse presented with only arthralgia,which was completely different from the initial manifestations.This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission,when clinical manifestations at relapse are different from those at onset.Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.

误诊为肺结核瘤的ANCA相关性血管炎临床分析

目的探讨抗中性粒细胞胞质抗体(ANCA)相关性血管炎的误诊原因及防范措施。方法回顾分析1例曾误诊的ANCA相关性血管炎的临床资料。结果本例因下肢红疹伴溃疡入院。曾行“鼻窦炎”手术,术前筛查发现肺部肿物行“肺部肿物切除术”,术后病理示肺结核瘤,抗结核治疗无效。入院后结合患者多系统受累表现及实验室、肾穿刺病理检查等诊断为ANCA相关性血管炎,给予糖皮质激素及调节免疫等治疗,症状缓解。结论ANCA相关性血管炎临床表现多样且不典型,易误诊。临床中应对疑似病例尽早行相关医技检查。

Animal models for anti-neutrophil cytoplasmic antibody-associated vasculitis:Are current models good enough?

Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multiple organ systems throughout the body.Our knowledge of the pathogenesis of AAV has increased considerably in recent years,involving cellular,molecular and genetic factors.Because of the controlled environment with no other confounding factors,animal models are beneficial for studying the mechanistic details of disease development and for providing novel therapeutic targets with fewer toxic side effects.However,the complexity and heterogeneity of AAV make it very difficult to establish a single animal model that can fully represent the entire clinical spectrum found in patients.The aim of this review is to overview the current status of animal models for AAV,outline the pros and cons of methods,and propose potential directions for future research.

Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schnlein purpura presenting mainly with gastrointestinal symptoms

AIM: To test the clinical significance of antineutrophil cytoplasmic antibody (ANCA) in evaluation of adult Henoch-Schnlein purpura (HSP) patients presenting mainly with abdominal symptoms. METHODS: Twenty-eight consecutive HSP patients who presented predominantly with abdominal symptoms were enrolled in this study. Control subjects included 27 age-and sex-matched patients with peptic ulcer disease,colon cancer,acute gastroenteritis,irritable bowel syndrome and colonic polyps. ANCA was measured by indirect immunofluorescence (ⅡF) in all patients,and follow-up ELISA was performed in patients with positive ⅡF tests. RESULTS: ANCA was detected in 9 HSP patients by ⅡF (2 were positive for c-ANCA and 7 were positive for p-ANCA). No ANCA was found in the control group. The sensitivity and specifi city of a positive ANCA test (either c-or p-ANCA) were 32.1% and 100% respectively. Only one out of the 9 patients with positive ANCA by ⅡF had positive ANCA by ELISA and the antigen was myeloperoxidase (MPO). The patients positive for ANCA had higher HSP clinical scores,and were more likely to have renal function impairment. Patients with late purpura development were also associated with more severe clinical manifestations. CONCLUSION: A positive ANCA test is associated with more severe symptoms in HSP. After inflammatory bowel disease is excluded,a positive ANCA test provides a clue to the diagnosis of HSP presenting predominantly with abdominal symptoms.

Antineutrophil cytoplasmic antibody associated vasculitides with renal involvement: Open challenges in the remission induction therapy

Renal involvement with rapidly progressive glomeru-lonephritis is a common manifestation of antineutrophil cytoplasmic antibody(ANCA) associated vasculitides, which is characterized by end-stage renal disease and high mortality rates in untreated and/or late referral patients. The long-term renal survival has improved dramatically since the addition of cyclophosphamide(CYC) and recently of rituximab(RTX) in association with corticosteroids in the remission induction thera-peutic regimens. However, renal prognosis remains unfavorable for many patients and the mortality rate is still significantly high. In this review, we analyze the open challenges to be addressed to optimize the induction remission therapy, principally in patients with advanced kidney failure. This concern the first-line therapy(CYC or RTX) based on different parameters(estimated glomerular filtration rate at baseline, new or relapsed disease, ANCA specificity, tissue injury, safety), the role of plasma exchange and the role of new therapies. Indeed, we discuss future perspectives in induction remission therapy by reporting recent advances in new targeted therapies with particular reference to avacopan, an orally administered selective C5a receptor inhibitor.

A subset of ulcerative colitis with positive proteinase-3 antineutrophil cytoplasmic antibody

A small subset of patients with active ulcerative colitis is non-responsive to major known non-biological therapies. We reported 5 patients with positive serum proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA) and tried to (1) identify the common clinical features of these patients; (2) investigate the efficacy of a novel therapy using a Chinese medicine compound; and (3) attract more gastroenterologists to be engaged in further study of this subset of patients. The common manifestations of disease in these 5 patients included recurrent bloody diarrhea and inflammatory lesions involving the entire colorectal mucosa. Initial treatment with intravenous methylprednisolone successfully induced remission. Four of these 5 patients were steroid-dependence, and immunosuppressants, such as azathioprine and cyclophosphamide, were in effective. In 3 patients, only the particular Chinese medicine compound could induce and maintain remission. One patient underwent colectomy. No vascular inflammatory lesions were found by histopathological examination. Although more cases are needed for confirmation, our study indicates thatulcerative colitis with positive PR3-ANCA may belong to a subtype of refractory ulcerative colitis. The particular Chinese medicine compound used in our study is by far the most effective in the management of these patients, with additional advantages of having no noticeable side-effects and less financial burden.

The role of mycophenolate in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis

Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis of guanosine nucleotides leading to selective inhibition of lymphocyte proliferation. Mycophenolic acid has been evaluated as induction and remission maintenance agent in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Since the course of disease of AAV usually requires long term immunosuppression, mycophenolate has been explored as a less toxic agent compared to cyclophosphamide and azathioprine. Mycophenolate is a potent immunosuppressive agent in the therapy of AAV, non-inferior to other available drugs with comparable side effect profile. Therefore, it could be a valuable alternative in cases of toxicity with life threatening side effects or intolerance to cyclophosphamide or azathioprine, in cases with high cumulative dose of cyclophosphamide, but also in cases with insufficient response. Several studies have shown a higher relapse rate following discontinuation of mycophenolate or in mycophenolate treated subjects that raises concerns about its usefulness in the treatment of AAV. This review describes the efficacy of mycophenolate in AAV as remission induction agent, as remission maintenance agent, and as therapeutic option in relapsing AAV disease, the relapse rate following discontinuation of mycophenolate, and the adverse events related to mycophenolate treatment.

血清同型半胱氨酸与胱抑素C联合检测对ANCA相关性肾损伤的诊断价值

目的 研究抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody, ANCA)相关性血管炎(ANCA-associated vasculitis, AAV)相关性肾损伤患者血清中同型半胱氨酸(HCY)与胱抑素C(Cys-C)的水平,探讨血清HCY和Cys-C联合检测对ANCA相关性肾损伤的诊断价值。方法 选取本院2016年1月—2019年1月肾内科收治的70例ANCA阳性合并肾损伤的患者,另选择同期前来就诊的48例ANCA阳性但并未合并肾损伤的患者(单纯ANCA阳性组)和50例体检的健康者(对照组)。比较各组检测结果并确定各检测指标对ANCA阳性合并肾损伤的受试者工作特征(ROC)曲线的曲线下面积(AUC),计算HCY、Cys-C联合检测对ANCA相关性肾损伤的相关性及诊断价值。结果 ANCA阳性合并肾损伤组、单纯ANCA阳性组与对照组中血清HCY、Cys-C水平差异有统计学意义(P<0.05);各组HCY、Cys-C的阳性率差异有统计学意义(P<0.05)。HCY、Cys-C联合诊断对ANCA阳性相关性肾损伤的诊断灵敏度、特异度高于上述指标单独检测,差异有统计学意义(P<0.05);ROC曲线结果显示,HCY联合Cys-C对ANCA阳性合并相关性肾损伤的患者具有较好的诊断价值和较高的相关性。结论 HCY与Cys-C联合检测与AAV相关性肾损伤密切相关,联合检测对ANCA阳性相关性肾损伤患者的诊断有积极作用,对患者治疗具有指导价值。

超小剂量利妥昔单抗治疗ANCA相关性血管炎一例

本文报道一例肺、肾受累的抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibodies, ANCA)相关性血管炎患者,给予经典的糖皮质激素、环磷酰胺、血浆置换诱导缓解治疗有效。该患者在使用硫唑嘌呤维持治疗期间共出现2次感染,后换用超小剂量(100 mg/次)利妥昔单抗维持治疗病情稳定。个体化利妥昔单抗治疗ANCA相关性血管炎效果显著,同时避免了过度免疫抑制,减轻了患者的经济负担。本例患者的诊疗经验有助于提高临床医生在ANCA相关性血管炎维持治疗方面的认识。

Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis:a nationwide retrospective study in China

Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis featured by liability to renal involvement.However,there are few studies on the risk factors and predictive models for renal outcomes of AAV in children.Methods Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospectively.The risk factors and predictive model of end-stage renal disease(ESRD)in AAV were explored.Results Renal involvement was the most typical manifestation(95.5%),and the crescent was the predominant pathological lesion(84.9%).The estimated glomerular filtration rate(eGFR)was evaluated in 114 patients,of whom 59.6%developed ESRD,and the median time to ESRD was 3.20 months.The eGFR[P=0.006,odds ratio(OR)=0.955,95%confidence interval(CI)=0.924–0.987]and the percentages of global glomerulosclerosis(pGGS;P=0.018,OR=1.060,95%CI=1.010–1.112)were independent risk factors for ESRD of renal biopsy.Based on the pGGS and eGFR at renal biopsy,we developed three risk grades of ESRD and one predictive model.The Kaplan‒Meier curve indicated that renal outcomes were significantly different in different risk grades(P<0.001).Compared with serum creatinine at baseline,the predictive model had higher accuracy(0.86 versus 0.58,P<0.001)and a lower coefficient of variation(0.07 versus 0.92)in external validation.Conclusions Renal involvement is the most common manifestation of pediatric AAV in China,of which more than half deteriorates into ESRD.The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children.

Autoimmune liver disease-related autoantibodies in patients with biliary atresia

AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies(ANAs), by line-blot assay; ANA and antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA(i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient.RESULTS The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls(14.8% vs 2.2%, P < 0.05). Accordingly, 32(25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(P < 0.05), with 6/92(8.6%) of patients with other liver diseases and 2/48(4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls(3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs(ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls(37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis(r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis.CONCLUSION High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.

Anti-Neutrophil Cytoplasmic Antibody Vasculitis in Pediatric Patients: Is the Incidence Rising?

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.

rare type of pancreatitis as the first presentation ofanti-neutrophil cytoplasmic antibody-related vasculitis

A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ultrasound-guided fine needle aspiration biopsy and endoscopic retrograde cholangiopancreatography failed to provide a definitive diagnosis. Based on the trend of improvement of the pancreatic enlargement, the treatment plan involved follow-up examinations. Later, he was hospitalized with an alveolar hemorrhage and rapidly progressive glomerulonephritis; he tested positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody(ANCA) and was diagnosed with ANCArelated vasculitis, specifically microscopic polyangiitis. It appears that factors such as thrombus formation caused by the vasculitis in the early stages of ANCArelated vasculitis cause abnormal distribution of the pancreatic blood flow, resulting in non-uniform pancreatitis. Pancreatic lesions in ANCA-related vasculitis are very rare. Only a few cases have been reported previously. Therefore, we report our case and a review of the literature.

血浆置换联合激素冲击治疗对ANCA相关性血管炎肾损伤患者临床实验室指标水平的影响

目的探讨血浆置换联合激素冲击治疗对抗中性粒细胞胞质抗体(ANCA)相关性血管炎肾损伤患者临床实验室指标水平的影响。方法选取2019年9月至2021年10月在空军军医大学第二附属医院肾内科接受治疗并确诊为ANCA相关性血管炎肾损伤的患者共40例,按照随机数字表法分为研究组和对照组,每组各20例。研究组患者采用血浆置换联合激素冲击治疗。对照组患者采用激素冲击治疗。比较两组治疗前后的实验室指标水平,包括血清免疫球蛋白G(IgG)、清蛋白(ALB)、肾功能指标[血清肌酐(Scr)、尿素氮(BUN)、尿沉渣红细胞计数(URBC)、24 h尿蛋白定量]、炎症指标[降钙素原(PCT)、C反应蛋白(CRP)]、血常规[血红蛋白(Hb)、血小板计数(PLT)]、凝血指标[活化部分凝血活酶时间(APTT)、凝血酶时间(TT)、纤维蛋白原(Fib)]。结果两组治疗后IgG、Scr、BUN水平均较治疗前明显降低,差异有统计学意义(P<0.05)。研究组治疗后URBC水平较治疗前明显降低,差异有统计学意义(P<0.05)。研究组治疗前后ALB水平比较,差异无统计学意义(P>0.05);而对照组治疗后ALB水平较治疗前降低,差异有统计学意义(P<0.05)。对照组治疗后APTT水平较治疗前升高,差异有统计学意义(P<0.05);研究组治疗后Fib水平高于对照组,且研究组治疗后Fib水平低于治疗前,差异有统计学意义(P<0.05)。结论血浆置换联合激素冲击治疗ANCA相关性血管炎肾损伤安全、有效,可改善患者肾功能,值得在临床工作中进一步推广使用。

儿童ANCA相关性血管炎合并肾损害七例临床分析

目的研究儿童抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)合并肾损害的临床特点、病理变化和及其治疗效果。方法回顾性分析2018年11月至2023年3月海南省妇女儿童医学中心肾病风湿免疫科收治的7例AAV患儿的临床特点、病理检查结果和诊治资料。结果7例AAV患儿均有肺部受损(其中1例弥漫性肺泡出血,1例肺含铁血黄素沉积症,1例呈白肺样改变),6例有肾脏受累(其中1例进展为严重的硬化性肾小球肾炎,2例为不同程度肾小球和肾小管损害)。7例患儿中4例予糖皮质激素(GC)联合免疫抑制剂治疗,2例予GC联合免疫抑制剂、利妥昔单抗(RTX)治疗,1例予GC联合RTX治疗。最终6例有不同程度好转,1例因肾功能不全于家中突发严重高钾血症,心跳呼吸骤停死亡。结论AAV合并肾损害并非少见,临床医师应及早完善ANCA和肾活检明确诊断,早期予GC联合生物制剂治疗的效果优于GC联合免疫抑制剂治疗。

抗中性粒细胞胞质抗体相关性肥厚性硬脑膜炎患者临床特点及影像学分析

目的分析抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性肥厚性硬脑膜炎(hypertrophic pachymeningitis,HP)患者的临床特点。方法回顾性收集2015年8月至2022年6月作者单位收治的10例ANCA相关性HP患者,其中来自中南大学湘雅医院9例,陕西省人民医院1例,男、女各5例,发病年龄43~80岁。分析患者的临床特征、影像学特点、诊治方法及疗效。结果10例患者表现为不同程度的慢性头痛或脑神经受累症状,病程1~84个月。5例患者血清髓过氧化物酶(myeloperoxidase,MPO)和核周型ANCA(perinuclear ANCA,p-ANCA)同时阳性,2例患者仅血清MPO阳性,p-ANCA和细胞质型ANCA(cytoplasmic ANCA,c-ANCA)阳性各1例,p-ANCA和c-ANCA同时阳性1例。6例视力受累,2例听力受累。增强MRI显示患者不同程度的硬脑膜增厚并强化,其中弥漫性增厚4例,局灶性增厚6例,均未累及脑实质。1例患者单用糖皮质激素治疗,1例患者行糖皮质激素联合丙球治疗,8例患者糖皮质激素联合免疫抑制剂治疗,其中1例同时使用利妥昔单抗,经治疗后患者症状均明显缓解。2例在激素和免疫抑制剂减量过程出现头痛加重,再次治疗后症状缓解。结论ANCA相关性HP病程长短不一,临床表现不典型,增强MRI显示的硬脑膜增厚部位在不同ANCA抗体阳性患者间有重叠;糖皮质激素和免疫抑制剂治疗ANCA相关性HP有效。

Antineutrophil cytoplasmic antibodies-associated glomerulonephritis:From bench to bedside

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders that pre-dominantly affects small vessels. The onset of the disease is closely associated with ANCA. Renal involvement, also known as ANCA-associated glomerulonephritis (AGN), is one of the most common manifestations of AAV. In this mini-review, we described the clinical and pathological features of AGN. We then focused on recent studies on the mechanism of acute kidney lesions, including fibrinoid necrosis and crescent formation. Following the basic aspects of kidney injury in AGN, we demonstrated the clinical importance of kidney injury in determining the outcome of patients with AGN. The prognostic value of the 2010 Histopathological Classification of AGN and validating studies were summarized. Finally, treatment and novel therapeutic strategies were introduced addressing the importance of optimizing management of this patient population.

肾脏疾病患者血清中ANCA的荧光模式及其意义

目的 :进一步探讨肾脏疾病患者血清中抗中性粒细胞细胞质抗体 (ANCA)的荧光模式及其意义。方法 :应用间接免疫荧光(IIF)和ELISA方法检测了 2 5 1例肾脏疾病患者血清中ANCA及其靶抗原。结果 :在 2 5 1例肾脏疾病患者中 ,ANCA阳性 5 1例占 2 0 .3% ,其中 10例为cANCA阳性 (10 / 2 5 1) ;30例为pANCA阳性 (30 / 2 5 1) ;11例为aANCA阳性 (11/ 2 5 1)。结论 :同时用IIF法和ELISA法检测ANCA ,可以提高ANCA检出的阳性率 ;不同ANCA荧光模式与疾病的种类病情及预后密切相关 ;提高对不典型ANCA和 pANCA伴ANA荧光模式的鉴别 。