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Dynamically changing antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis:A case report
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作者 Yan Zhang Qiao-Ding Dai +3 位作者 Ji-An Wang Li-Ping Xu Qiang Chen Yang-Zi Jin 《World Journal of Clinical Cases》 SCIE 2024年第16期2881-2886,共6页
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 granulomatou... 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. 展开更多
关键词 Anti-neutrophil cytoplasmic antibodies Granulomatosis with polyangiitis antineutrophil cytoplasmic antibodyassociated vasculitis Immunosuppressive therapy Case report
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Antineutrophil cytoplasmic antibodies crescentic allograft glomerulonephritis after sofosbuvir therapy 被引量:2
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作者 Shilpa Gadde Belinda Lee +1 位作者 Laura Kidd Rubin Zhang 《World Journal of Nephrology》 2016年第6期547-550,共4页
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... 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. 展开更多
关键词 Crescentic glomerulonephritis VASCULITIS antineutrophil cytoplasmic antibody Sofosbuvir Kidney transplant
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A subset of ulcerative colitis with positive proteinase-3 antineutrophil cytoplasmic antibody 被引量:2
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作者 Jin Xu Chuan-Hua Yang +3 位作者 Xiao-Yu Chen Xu-Hang ki Min Dai Shu-Dong Xiao 《World Journal of Gastroenterology》 SCIE CAS CSCD 2008年第45期7012-7015,共4页
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-ANC... 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. 展开更多
关键词 Refractory ulcerative colitis Proteinase-3 antineutrophil cytoplasmic antibody Methyprednisolone Steroid-dependence Chinese medicine
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Perinuclear anti-neutrophil cytoplasmic antibodies (p-anca) in chronic ulcerative colitis: Experience in a Mexican institution 被引量:3
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作者 Jesus K Yamamoto-Furusho Takeshi Takahashi-Monroy +2 位作者 Omar Vergara-Fernandez Edgardo Reyes Luis Uscanga 《World Journal of Gastroenterology》 SCIE CAS CSCD 2006年第21期3406-3409,共4页
AIM: To assess the prevalence and clinical value of p-ANCA in a sample of Mexican ulcerative colitis (UC) patients. METHODS: In a prospective, IRB-approved protocol, p-ANCA was determined in 80 patients with UC (... AIM: To assess the prevalence and clinical value of p-ANCA in a sample of Mexican ulcerative colitis (UC) patients. METHODS: In a prospective, IRB-approved protocol, p-ANCA was determined in 80 patients with UC (mean age, 32 ± 12.9 years). The severity and extension of disease were determined by clinical methods, searching a statistical association with p-ANCA status. RESULTS: p-ANCA were detected in 41 (51%) patients. Severity of disease was the only clinical variable statistically associated with their presence (P 〈 0.0001; OR = 9; CI 95% = 3.2-24.7). CONCLUSION: The prevalence of p-ANCA was similar to that reported in other countries. Their presence was associated to UC severity, but offered no more information than the obtained by clinical methods. 展开更多
关键词 Ulcerative colitis Inflammatory bowel disease Perinuclear anti-neutrophil cytoplasmic antibodies
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CLINICAL AND PATHOLOGICAL MANIFESTATI-ONS OF PATIENTS WITH ANTINEUTROPHIL CYTO-PLASMIC AUTOANTIBODIES DIRECTED AGA INST PROTEINASE 3 OR MYELOPEROXIDASE 被引量:1
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作者 张? 董怡 +2 位作者 曾小峰 李永哲 唐福林 《Chinese Medical Sciences Journal》 CAS CSCD 2002年第1期32-35,共4页
To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinas e 3 (anti PR3) or myeloperoxidase (anti MPO). Methods. One hundred a... To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinas e 3 (anti PR3) or myeloperoxidase (anti MPO). Methods. One hundred and forty patients with ANCA were detected for anti PR3 a nd anti MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti P R3 or anti MPO were analysed.Results. In anti PR3 group (n=21), 16 cases (76.2%) had systemic vasculitis , in which Wegener’s granulomatosis prevailed (13 cases, 61.9%). In anti MPO g roup (n=31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 case s (38.7%) as microscopic angiitis. For vasculitic patients with anti PR3 and a nti MPO, the disease duration at diagnosis was 9.6±2.0m and 4.4±0.9m respecti vely, P< 0.05;vasculitis activity index (BVAS) and mean number of affected organ were 22.5±2.1, 5.0±0.4 and 25.1±1.7, 4.8±0.4 respectively, P >0.05;upper r espiratory tract, eye and joint involvements were 11(68.8%), 7(43.8%), 11(68.8 %) and 7(36.8%), 2(10.5%), 5(26.3%) respectively, P< 0.05.Although there was no statistical difference in renal involvement between these two groups, patien ts with serum creatine >500 μmol/L were more commonly seen in anti MPO group t han in anti PR3 group, which were 8(42.1%) and 2(12.5%) respectively, P< 0.05 . Ten relapses were seen in anti PR3 group and only 2 in anti MPO group, but t he acute mortality rate in anti MPO group (5/19, 27.4%) was much higher than t hat in anti PR3 group (1/16, 6.3%). Conclusions. Anti PR3 and anti MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti PR 3 and those with anti MPO. In particular, upper respiratory tract, eye and join t involvements, granuloma formation and relapse were more prominent in anti PR3 patients. By contrast, the anti MPO patients had a more acute disease onset, m ore rapid progressive renal involvement and a higher acute mortality rate. 展开更多
关键词 antineutrophil cytoplasmic autoantibodies MYELOPEROXIDASE proteina se 3
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Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schnlein purpura presenting mainly with gastrointestinal symptoms 被引量:10
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作者 Yan Zhang Yong-Kang Wu +1 位作者 Matthew A Ciorba Qin Ouyang 《World Journal of Gastroenterology》 SCIE CAS CSCD 2008年第4期622-626,共5页
AIM: To test the clinical significance of antineutrophil cytoplasmic antibody (ANCA) in evaluation of adult Henoch-Schonlein purpura (HSP) patients presenting mainly with abdominal symptoms. METHODS: Twenty-eigh... AIM: To test the clinical significance of antineutrophil cytoplasmic antibody (ANCA) in evaluation of adult Henoch-Schonlein 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 ageand 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 specificity 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. 展开更多
关键词 Henoch-Schonlein purpura antineutrophil cytoplasmic antibody Diagnosis Indirect immuno-fluorescence
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The role of mycophenolate in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis 被引量:2
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作者 Maria Koukoulaki Christos Iatrou 《World Journal of Nephrology》 2019年第4期75-82,共8页
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... 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. 展开更多
关键词 Mycophenolic acid MYCOPHENOLATE mofetil MYCOPHENOLATE sodium antineutrophil cytoplasmic antibody-associated VASCULITIS Microscopic polyangiitis GRANULOMATOSIS with polyangiitis induction REMISSION Relapse
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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 被引量:24
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作者 S Desplat-Jégo C Johanet +10 位作者 A Escande J Goetz N Fabien N Olsson E Ballot J Sarles JJ Baudon JC Grimaud M Veyrac P Chamouard RL Humbel 《World Journal of Gastroenterology》 SCIE CAS CSCD 2007年第16期2312-2318,共7页
AIM: Anti-Saccharomyces anti-nuclear associated cerevisiae antibodies (ASCA), anti-neutrophil antibodies (NANA) and antibodies to exocrine pancreas (PAB), are serological tools for discriminating Crohn's disea... AIM: Anti-Saccharomyces anti-nuclear associated cerevisiae antibodies (ASCA), 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. 展开更多
关键词 Inflammatory bowel disease Coeliac disease Anti-Saccharomyces cerevisiae antibodies Anti-neutrophil cytoplasmic antibodies Anti-pancreatic antibodies
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Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in a 77-year-old man: A case report 被引量:1
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作者 Zi-Gan Xu Wei-Long Li +6 位作者 Xi Wang Shu-Yuan Zhang Ying-Wei Zhang Xing Wei Chun-Di Li Ping Zeng Shao-Dong Luan 《World Journal of Clinical Cases》 SCIE 2021年第3期707-713,共7页
BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologi... BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury. 展开更多
关键词 Systemic lupus erythematosus antineutrophil cytoplasmic antibodyassociated vasculitis Overlap syndrome Elderly male Renal insufficiency Case report
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Olfactory dysfunction in antineutrophil cytoplasmic antibodyassociated vasculitides: A review of the literature
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作者 Alfonso Luca Pendolino Anika Kaura +6 位作者 Annakan V Navaratnam Monica Pendolino Gerolamo Bianchi Samit Unadkat Giancarlo Ottaviano Premjit S Randhawa Peter J Andrews 《World Journal of Methodology》 2021年第2期15-22,共8页
Olfactory dysfunction(OD)has been described in patients with antineutrophil cytoplasmic antibody-associated vasculitides(AAV),but the underlying mechanisms are not completely understood.The causes of altered smell fun... Olfactory dysfunction(OD)has been described in patients with antineutrophil cytoplasmic antibody-associated vasculitides(AAV),but the underlying mechanisms are not completely understood.The causes of altered smell function can generally be divided into conductive,sensorineural or others.To date no specific treatment is available for AAV-related OD and the efficacy of currently available options has not been explored.The aim of this review is to provide an overview of the causes that may lead to OD in patients with AAV.Current available treatments for OD and possible options in patients with AAV presenting with smell impairment are also mentioned. 展开更多
关键词 Smell Olfactory dysfunction antineutrophil cytoplasmic antibody-associated vasculitis diseases Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis Microscopic polyangiitis
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Autoimmune liver disease-related autoantibodies in patients with biliary atresia 被引量:7
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作者 Shu-Yin Pang Yu-Mei Dai +9 位作者 Rui-Zhong Zhang Yi-Hao Chen Xiao-Fang Peng Jie Fu Zheng-Rong Chen Yun-Feng Liu Li-Yuan Yang Zhe Wen Jia-Kang Yu Hai-Ying Liu 《World Journal of Gastroenterology》 SCIE CAS 2018年第3期387-396,共10页
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 contr... 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. 展开更多
关键词 BILIARY ATRESIA Anti-nuclear ANTIBODY antineutrophilic cytoplasmic ANTIBODY AUTOIMMUNE liver diseases AUTOantibodies
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Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis:a nationwide retrospective study in China
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作者 Li-Wen Tan Jun-Li Wan +24 位作者 Chun-Hua Zhu Hong Xu Zheng-Kun Xia Li-Zhi Chen Xiao-Chuan Wu Fang Wang Xiao-Rong Liu Cheng-Guang Zhao Xiao-Zhong Li Jian-Hua Mao Xiao-Wen Wang Wen-Yan Huang Yu-Hong Li Jian-Jiang Zhang Shi-Pin Feng Jun Yang Jiao-Jiao Liu Chun-Lin Gao Li-Ping Rong Lan-Jun Shuai Ke Xu He-Jia Zhang Qiu Li Ai-Hua Zhang Mo Wang 《World Journal of Pediatrics》 SCIE CSCD 2024年第5期506-516,共11页
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... 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. 展开更多
关键词 antineutrophil cytoplasmic antibody End-stage renal disease GLOMERULONEPHRITIS Pediatric nephrology VASCULITIS
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Antineutrophil cytoplasmic antibodies-associated glomerulonephritis:From bench to bedside 被引量:9
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作者 Yong-Xi Chen Xiao-Nong Chen 《Chronic Diseases and Translational Medicine》 CSCD 2018年第3期187-191,共5页
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 in... 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. 展开更多
关键词 antineutrophil cytoplasmic antibodies RENAL involvement Prognosis Treatment
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Myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis with headache and kidney involvement at presentation and with arthralgia at relapse:A case report
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作者 Xue Zhang Guang-Ben Zhao +3 位作者 Long-Kai Li Wei-Dong Wang Hong-Li Lin Ning Yang 《World Journal of Clinical Cases》 SCIE 2023年第21期5167-5172,共6页
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 rela... 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. 展开更多
关键词 antineutrophil cytoplasmic antibody associated vasculitis HEADACHE KIDNEY ARTHRALGIA RELAPSE Case report
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Significance of anti neutrophil cytoplasmic antibodies in patients with pauci immune crescentic glomerulonephritis
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作者 唐政 周虹 +4 位作者 张少陵 姚小丹 胡伟新 刘志红 黎磊石 《Chinese Medical Journal》 SCIE CAS CSCD 1999年第12期87-90,共4页
Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomeruloneph... Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity 展开更多
关键词 anti neutrophil cytoplasmic antibodies pauci immune crescentic glomerulonephritis
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Analysis of clinical features and prognosis of anti-glomerular basement membrane antibody positive patients with anti-neutrophil cytoplasmic antibodies
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作者 杨娟 《China Medical Abstracts(Internal Medicine)》 2017年第1期48-,共1页
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 gl... 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 展开更多
关键词 ANCA GBM Analysis of clinical features and prognosis of anti-glomerular basement membrane antibody positive patients with anti-neutrophil cytoplasmic antibodies
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Antineutrophil cytoplasmic antibody associated vasculitides with renal involvement: Open challenges in the remission induction therapy 被引量:1
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作者 Maurizio Salvadori Aris Tsalouchos 《World Journal of Nephrology》 2018年第3期71-83,共13页
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... 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. 展开更多
关键词 Rapidly progressive GLOMERULONEPHRITIS REMISSION induction therapy antineutrophil cytoplasmic antibody ASSOCIATED VASCULITIDES CYCLOPHOSPHAMIDE Rituximab CORTICOSTEROIDS Plasma exchange Avacopan
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Animal models for anti-neutrophil cytoplasmic antibody-associated vasculitis:Are current models good enough?
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作者 Fei Huang Yongman Lv +2 位作者 Siyang Liu Hao Wu Qingquan Liu 《Animal Models and Experimental Medicine》 CAS CSCD 2023年第5期452-463,共12页
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 multi... 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. 展开更多
关键词 antineutrophil cytoplasmic antibody experimental models PATHOGENESIS VASCULITIS
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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
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作者 陈晓莉 王群 +2 位作者 刘佳 李静 张国平 《中国神经免疫学和神经病学杂志》 CAS 2024年第1期40-45,共6页
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... 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。 展开更多
关键词 肥厚性硬脑膜炎 抗体 抗中性白细胞胞质 磁共振成像 过氧化物酶
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Murtagh安全诊断策略联合思维导图构建临床思维在全科教学门诊中的应用
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作者 杨玲 杜雪平 《中国全科医学》 CAS 北大核心 2025年第6期673-680,共8页
全科教学门诊是培养住院医师规范化培训(简称住培)全科医生临床思维、提升全科诊疗能力的重要培训方法。本文用1例以乏力为主要表现的患者为教学案例,介绍全科教学门诊中采用澳大利亚著名全科医学专家John Murtagh提出的Murtagh安全诊... 全科教学门诊是培养住院医师规范化培训(简称住培)全科医生临床思维、提升全科诊疗能力的重要培训方法。本文用1例以乏力为主要表现的患者为教学案例,介绍全科教学门诊中采用澳大利亚著名全科医学专家John Murtagh提出的Murtagh安全诊断策略启发住培全科医生对乏力进行诊断与鉴别诊断:(1)引起乏力的常见疾病有哪些?(2)哪些重要疾病是不能忽视的?(3)乏力有什么容易被遗漏的疾病?(4)是否存在潜在的容易被掩盖的疾病?(5)患者是否有话没有说?结合病史、体格检查和实验室检查结果初步诊断为抗中性粒细胞胞浆抗体相关性血管炎引起急进性肾小球肾炎可能性大,及时转诊,肾病理检查确诊乏力的病因为抗中性粒细胞胞浆抗体相关性肾小球肾炎,取得满意疗效。带教老师基于Murtagh安全诊断策略帮助住培全科医生构建乏力鉴别、分析的系统知识框架,从而提升住培全科医生的临床逻辑思维能力和解决临床实际问题的能力;以思维导图为辅助工具,将Murtagh安全诊断策略诊断与鉴别诊断思路可视化、形象化,优化教学效果。 展开更多
关键词 全科医学 Murtagh安全诊断策略 思维导图 全科临床思维 乏力 抗中性粒细胞胞浆抗体相关性肾小球肾炎
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