Perinuclear anti-neutrophil cytoplasmic antibodies (p-anca) in chronic ulcerative colitis: Experience in a Mexican institution

瞄准：在墨西哥 ulcerative (UC ) 的一件样品估计流行和 p-ANCA 的临床的价值病人。方法：在一个未来的、同意 IRB 的协议， p-ANCA 与 UC 在 80 个病人被决定(吝啬的年龄， 32 +/- 12.9 年) 。疾病的严厉和延期被临床的方法决定，寻找有 p-ANCA 地位的一个统计协会。结果：p-ANCA 在 41 被检测(51%) 病人。疾病的严厉是统计上与他们的存在联系的唯一的临床的变量(P 【 0.0001；或 = 9；CI 95%= 3.2-24.7 ) 。结论：p-ANCA 的流行类似于在另外的国家报导了那。他们的存在被联系到 UC 严厉，但是比提供了没有更多的信息由临床的方法获得了。

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.

Analysis of clinical features and prognosis of anti-glomerular basement membrane antibody positive patients with anti-neutrophil cytoplasmic antibodies

Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM glomerulonephritis patients were collected and were tested for ANCA respectively.Characteristics and outcome of patients with coexisting anti-GBM antibody

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.

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.

Update on Anti-Saccharomyces cerevisiae antibodies, anti-nuclear associated anti-neutrophil antibodies and antibodies to exocrine pancreas detected by indirect immunofluorescence as biomarkers in chronic inflammatory bowel diseases: Results of a multicent

AIM: Anti-Saccharomyces cerevisiae antibodies (ASCA), anti-nuclear associated anti-neutrophil antibodies (NANA) and antibodies to exocrine pancreas (PAB), are serological tools for discriminating Crohn’s disease (CrD) and ulcerative colitis (UC). Like CrD, coeliac disease (CoD) is an inflammatory bowel disease (IBD) associated with (auto) antibodies. Performing a multicenter study we primarily aimed to determine the performance of ASCA, NANA and PAB tests for IBD diagnosis in children and adults, and secondarily to evaluate the prevalence of these markers in CoD. METHODS: Sera of 109 patients with CrD, 78 with UC, 45 with CoD and 50 healthy blood donors were retrospectively included. ASCA, NANA and PAB were detected by indirect immunofluorescence (IIF). RESULTS: ASCA+/NANA- profile displayed a positive predictive value of 94.2% for CrD. Detection of ASCA was correlated with a more severe clinical profile of CrD and treatment of the disease did not influence their serum levels. ASCA positivity was found in 37.9% of active CoD.PAB were found in 36.7% CrD and 13.3% CoD patients and were not correlated with clinical features of CrD, except with an early onset of the disease. Fifteen CrD patients were ASCA negative and PAB positive. CONCLUSION: ASCA and PAB detected by IIF are specific markers for CrD although their presence does not rule out a possible active CoD. The combination of ASCA, NANA and PAB tests improves the sensitivity of immunological markers for CrD. Repeating ASCA, NANA, and PAB testing during the course of CrD has no clinical value.

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.

Differentiation of Behcet's disease from inflammatory bowel diseases:Anti-saccharomyces cerevisiae antibody and anti-neutrophilic cytoplasmic antibody

The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and anti-neutrophilic cytoplasmic antibody(p-ANCA) in the differential diagnosis of BD from IBD.

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.

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.

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.

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。

他克莫司、吗替麦考酚酯联合糖皮质激素治疗抗中性粒细胞胞质抗体相关性血管炎肾损害的效果

目的比较他克莫司(tacrolimus,TAC)、吗替麦考酚酯(mycophenolate mofetil,MMF)联合糖皮质激素治疗抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性血管炎(ANCA-associated vasculitis,AAV)肾损害的效果。方法选取医院收治的AAV患者80例,用随机数字表法分为A组(n=41,予以TAC联合糖皮质激素治疗)和B组(n=39,予以MMF联合糖皮质激素治疗)。比较2组患者ANCA、伯明翰血管炎活动性评分(Birmingham vasculitis activity score,BVAS)、肾功能及血管内皮功能相关指标、细胞免疫指标、不良反应。结果治疗后,2组的ANCA、BVAS、24 h尿蛋白定量(24-hour urinary pro⁃tein quantification,24 h UPQ)、尿素氮(blood urea nitrogen,BUN)、胱抑素C(cystatin C,CysC)、可溶性血管内皮细胞生长因子受体1(soluble fms-like tyrosine kinase-1,SFlt-1)、溶酶体相关膜蛋白2(recombinant lysosomal associated membrane protein 2,LAMP-2)抗体、血管内皮细胞生长因子(vascular endothelial growth factor,VEGF)均降低(P<0.05),且A组较B组低(P<0.05);2组的CD4+均升高,A组较B组更高(P<0.05);2组的CD8+、CD19+均降低,A组较B组更低(P<0.05)。A组的不良反应总发生率(9.76%)较B组(28.21%)低(P<0.05)。结论与MMF联合糖皮质激素治疗方案比较,TAC联合糖皮质激素治疗AAV肾损害在降低血清ANCA值、减小BVAS、改善肾功能、保护血管内皮功能、调节细胞免疫等方面的效果更加显著,且安全性高。

集束化护理在ANCA相关性血管炎肾损害患者中的应用

目的探讨集束化护理在抗中性粒细胞胞质抗体(ANCA)相关性血管炎肾损害患者中的应用效果。方法前瞻性选取2022年1月至2023年6月郑州大学第一附属医院收治的110例ANCA相关性血管炎肾损害患者作为研究对象,按随机数表法分为观察组和对照组各55例。对照组患者采用常规护理,观察组患者在常规护理的基础上给予集束化护理。两组患者均护理至出院,比较两组患者护理前和出院时的血压、平均动脉压、血红蛋白(Hb)和C反应蛋白(CRP)水平和肾功能恢复情况[包括尿素氮(BUN)、血清肌酐(CR)、尿β_(2)-微球蛋白(β_(2)-MG)、胱抑素C(Cys-C)、肾小球滤过率(GFR)情况];比较两组患者护理前和出院时的汉密尔顿抑郁量表(HAMD)评分变化;出院时,统计两组患者的不良反应发生率,并采用自制调查问卷调查两组患者出院时的护理满意度。结果出院时,两组患者的收缩压、舒张压、平均动脉压、Hb、CRP均下降,且观察组明显低于对照组,差异均有统计学意义(P<0.05);出院时,两组患者的Cys-C、GFR均升高,且观察组患者的Cys-C、GFR分别为(792.12±43.83)μg/L、(33.32±4.36)g/L,明显高于对照组的(666.94±57.94)μg/L、(32.34±4.35)g/L,BUN、CR、β_(2)-MG均降低,且观察组患者的BUN、CR、β_(2)-MG分别为(4.02±1.33)mmol/L、(328.25±120.55)mmol/L、(0.14±0.04)mmol/L,明显低于对照组的(4.95±2.73)mmol/L、(420.42±130.38)mmol/L、(0.44±0.04)mmol/L,差异均有统计学意义(P<0.05);出院时,两组患者的HAMD评分均降低,且观察组患者的HAMD为(13.24±1.05)分,明显低于对照组的(26.24±3.19)分,差异均有统计学意义(P<0.05);观察组患者的不良反应发生率为10.91%,明显低于对照组的43.64%,护理满意度为98.18%,明显高于对照组的70.91%,差异均有统计学意义(P<0.05)。结论集束化护理应用于ANCA相关性血管炎肾损害患者中,能够控制患者的血压,改善肾功能,改善患者抑郁情绪,同时有助于减少不良反应发生率,提高患者满意度。

血小板/中性粒细胞比值在ANCA相关性血管炎疾病活动及预后评估中的临床价值

目的评估血小板/中性粒细胞比值(PNR)在抗中性粒细胞胞浆抗体(ANCA)相关血管炎疾病活动度及预后的价值。方法回顾性分析2015年3月至2023年7月于我院经肾活检确诊为AAV的128例患者临床资料。采用Spearman法进行相关性分析,通过t检验或秩和检验对比组间差异,Kaplan-Meier生存分析和Cox比例风险回归模型分析患者预后。结果PNR与伯明翰血管炎活动性评分(BVAS)(r=-0.268,P=0.002)负相关,PNR水平对疾病活动的预测效能的ROC曲线下面积(AUC)为0.672。根据PNR的最佳截点值(26.4)将患者分为高水平组(PNR≥26.4)(n=105)和低水平组(PNR<26.4)(n=23)。与低水平PNR组相比,高水平组患者预后情况明显优于低水平组(P<0.001),PNR与肾脏预后不良独立相关(OR=2.54,95%CI:1.1-5.88,P=0.029)。结论PNR降低提示AAV活动性增强及预后不良,有望成为评估活动性及预测预后的生物标志物。

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。