抗中性粒细胞胞质抗体阳性的感染性心内膜炎18例临床分析

目的通过分析ANCA阳性的感染性心内膜炎(IE)的临床特点, 旨在提高临床诊疗水平。方法收集2016年6月至2021年7月在郑州大学第一附属医院住院确诊为IE且同时伴有血清学ANCA阳性的18例患者的临床资料, 回顾性分析其一般情况、临床表现、实验室检查、影像学检查、治疗及转归。采用SPSS 20.0软件进行统计分析。计数资料以例数和百分比(%)表示, 计量资料以±s表示。结果 18例患者中, 男性12例, 女性6例, 平均发病年龄(50±16)岁。除2例为髓过氧化物酶(MPO)-ANCA阳性外, 余均为蛋白酶3(PR3)-ANCA阳性。主要临床表现有发热(88.9%, 16/18)、贫血(72.2%, 13/18)、脾大(44.4%, 8/18)、心脏杂音(33.3%, 6/18)、关节痛(22.2%, 4/18)、肝损伤(22.2%, 4/18)、栓塞事件(16.7%, 3/18)、Osler结节(11.1%, 2/18)和肾功能不全(11.1%, 2/18)。83.3%(15/18)的患者ESR、CRP和降钙素原(PCT)明显升高。血培养阳性率50.0%(9/18), 链球菌(7...     

老年抗中性粒细胞胞浆抗体相关性小血管炎肺部损害29例临床分析

目的 探讨老年抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏损害的临床特征.方法 分析2002年9月至2011年9月住院的29例60岁以上的ANCA相关性小血管炎肺损害患者的临床资料.结果 29例ANCA相关性小血管炎肺脏损害患者.常见症状有咳嗽、咯痰(19/29,65.5%);呼吸困难(14/29,48.2%);咯血(7/29,24.1%)和发热(16/29,55.1%).所有患者ANCA均阳性,其中髓过氧化物酶(MPO)-ANCA阳性率89.7%(26/29),蛋白酶3(PR3)-ANCA阳性率10.3%(3/29).胸部影像学表现为肺间质纤维化16例,磨玻璃影者6例,浸润影5例,结节性病变1例,胸腔积液1例.24例(82.8%)合并肾脏受累表现.经激素和免疫抑制剂治疗后短期预后较好,9例(31%)于初诊及随访期间因肺部感染呼吸衰竭等死亡.结论 老年原发性ANCA相关性小血管炎患者常有肺脏损害,其临床表现无特异性,肺间质纤维化、磨玻璃影为常见影像学表现,且多合并肾脏受累.     

抗中性粒细胞胞质抗体阳性的感染性心内膜炎18例临床分析

目的:通过分析ANCA阳性的感染性心内膜炎（IE）的临床特点,旨在提高临床诊疗水平。方法:收集2016年6月至2021年7月在郑州大学第一附属医院住院确诊为IE且同时伴有血清学ANCA阳性的18例患者的临床资料,回顾性分析其一般情况、临床表现、实验室检查、影像学检查、治疗及转归。采用SPSS 20.0软件进行统计分析。...     

血清抗中性粒细胞胞质抗体阳性狼疮性肾炎患者的临床病理特征及预后

目的:回顾性分析血清抗中性粒细胞胞质抗体(ANCA)阳性狼疮性肾炎(LN)患者的临床、病理特征及预后。方法:收集本中心1985年1月至2008年12月间明确诊断为血清ANCA阳性LN患者,回顾性分析其临床、病理特征及预后,对照组选取同期诊断为血清ANCA阴性LN患者。结果:符合入选标准的49例患者中,38例MPO-ANCA阳性,7例PR3-ANCA阳性,4例MPO-ANCA和PR3-ANCA双阳性。女性40例、男性9例。中位年龄33岁。ANCA阳性LN患者,尿沉渣红细胞计数、血清肌酐、SLE-DAI评分均高于对照组(P0.05);病理类型以LN-Ⅳ型为主,占61.22%;光镜下AI、CI评分均显著高于对照组(P0.05)。ANCA阳性LN患者中,20例接受吗替麦考酚酸酯(MMF)诱导治疗,18例接受静脉环磷酰胺(CYC)诱导治疗,其余11例患者接受雷公藤多苷等治疗MMF组的完全缓解率(90.00%vs 55.56%,P=0.027)和肾脏生存率(P=0.039)均优于CYC组。ANCA阳性LN患者中位随访87.83月,有7例患者(14.29%)发生终末期肾病,均为MPO-ANCA阳性。结论:血清ANCA阳性LN患者肾脏表现为大量的血尿、肾功能损害明显。这部分患者接受MMF诱导治疗,其完全缓解率和肾脏预后均优于CYC。     

感染性心内膜炎120例临床分析

目的 探讨感染性心内膜炎(IE)的基础病因、致病微牛物和临床特征,提高IE的诊治水平.方法 回顾件分析北京协和医院1997年10月-2007年9月确诊的120例IE患者的临床资料.结果 120例IE患者中,108例(90.0%)为自然瓣膜心内膜炎(NVE),12例(10.0%)为人工瓣膜心内膜炎(PVE);29例(24.2%)无基础心脏病变,79例(73.1%)NVE患者存在各种基础心脏病,其中先天性心血管畸形30例(38.0%),其次为特发性二尖瓣脱垂(23例,29.1%)和风湿性心脏病(11例,13.9%).临床主要表现为发热(100.0%)、贫血(65例,54.2%)和栓塞(58例,48.3%).有83例(69.2%)细菌培养阳性,其中43例(51.8%)致病菌为链球菌.结论 IE最常见的基础心脏病前3位是先天性心血管畸形、二尖瓣脱垂和风湿性心脏病.不明原因长期发热患者应想到心内膜炎?鬃 的町能,血培养和超声心动图检查有助于IE的诊断.     

肥厚型梗阻性心肌病合并感染性心内膜炎14例临床分析

目的探讨肥厚型心肌病合并感染性心内膜炎(IE)病例的临床特征。方法 2006年8月至2018年6月阜外医院收治肥厚型心肌病(HCM)患者7 427例, IE患者668例,其中HCM合并IE者14例,回顾性分析其临床特征,描述14例患者的临床表现、病原学特征、超声心动图发现、治疗及出院转归等。结果 HCM合并IE发生率为0.19%,在HCM患者中IE的发病率为0.15/1 000人年;HCM合并IE 14例患者中男性占11例,发热及心脏杂音是最常见的临床表现;心功能不全(12/14)及细菌性栓塞(8/14)为最常见并发症;致病微生物均为革兰阳性球菌,以链球菌最常见(5/8);超声心动图提示室间隔厚度(21.2±2.7)mm,左心室流出道梗阻重,14例均有赘生物形成,赘生物最多分布于二尖瓣前叶(12/14),体循环栓塞(8/14)及瓣叶损害(12/14)发生率高;好转出院10例,其中8例为接受外科手术治疗者;2例自动出院后死亡,2例院内死亡。结论 HCM合并IE发生率低,HCM合并IE患者细菌性栓塞及瓣叶损害的发生率较高,且住院病死率高,预后较差,外科手术是治疗HCM合并IE成功的基石。     

感染性心内膜炎97例临床分析

目的 了解近年来感染性心内膜炎(IE)的临床特点变化.方法 回顾性分析华山医院近10年收治的97例IE患者的临床资料,包括发病年龄、易感因素、临床表现、血培养及超声心动图检查.计量数值如患者发病年龄等以算术平均数±标准差表示,病例阳性数以百分比表示,对比分析采用Fisher确切概率法.结果 97例IE患者平均年龄(49±17)岁,有基础心脏病73例,占75.3％;前三位是风湿性心脏病27例(27.8％)、先天性心脏病23例(23.7％)、特发性二尖瓣脱垂18例(18.6％).临床表现为发热96例(99.0％)、心脏杂音93例(95.9％)、贫血82例(84.5％).血液细菌培养阳性66例(68.0％),其中草绿色链球菌28株(42.4％),仍为主要致病菌,且全部发生于自然瓣膜心内膜炎患者中;葡萄球菌18株(27.3％),金黄色葡萄球菌10株(15.2％),有3株为耐甲氧西林金黄色葡萄球菌(MRSA),凝固酶阴性葡萄球菌8株(12.1％),有2株为耐甲氧西林凝固酶阴性葡萄球菌(MRCNS);鲍曼不动杆菌、嗜麦芽窄食单胞菌等耐药性强的病原菌各1株.超声心动图赘生物检出79例,占81.4％,其中右心赘生物9例,病原主要为葡萄球菌.结论 IE患者临床表现、易感因素、致病菌发生了变化,多次血培养及超声心动图检查有助于诊断.     

单纯右心感染性心内膜炎18例临床分析

目的 回顾性分析18例单纯右心感染性心内膜炎(RSIE)的临床特点,提高对该病的认识水平.方法 对2005年8月至2009年2月于我院住院治疗的18例RSIE患者的临床及流行学特点进行回顾性分析.结果 本组18例RSIE,占同时期住院的感染性心内膜炎总数的7.23%(18/249).基础病因中先天性心脏病居首位,占76.5%(14/18),其中室间隔缺损最为常见;心脏手术后3例,均于术后早期(＜60 d)发生RSIE;无基础心脏病1例,但有大剂量糖皮质激素应用史.发热(＞1周)是最常见的临床表现(100%,18/18),细菌性肺动脉栓塞5例,是最常见的并发症.血培养阳性率偏低(38.9%,7/18),金黄色葡萄球菌是最常见致病菌(4/7).18例(100%)经胸超声心动图均有阳性发现,其中17例发现赘生物共22处,l3处发生于三尖瓣.本组患者无院内死亡,未愈出院2例,好转10例,治愈6例;平均住院(22.0±18.9)d.结论 本组患者的基础病因以先天性心脏病占有很大比例,而非先天性心脏病RSIE多为院内获得性感染.金黄色葡萄球菌是RSIE最常见的致病菌.超声心动图是诊断IE的重要工具.     

感染性心内膜炎33例临床分析

本文总结分析了33例感染性心内膜炎(IE)的临床特点:其基础心脏病风心、先心及正常心脏分别为51.5%(17/13),30.3%(10/33)和21.2%(7/33)。发热及栓塞为主要临床表现,超声心动图(UCG)赘生物检出率高达(70.4%),为诊断IE的重要手段。     

长期发热的感染性心内膜炎35例临床分析

目的:分析感染性心内膜炎的临床特征,提高感染性心内膜炎的诊断意识.方法:回顾分析我院近13年35例感染性心内膜炎的患者的临床特征及诊断.结果:①35例患者中33例为自体心脏瓣膜心内膜炎,2例为人工瓣膜心内膜炎.33例自体瓣膜心内膜炎中,基础心脏病占78.8%(26/33),其中先天性心脏病占53.8%(14/26)、原发性二尖瓣脱垂占26.9%(7/26)、风湿性心脏病占19.2%(5/26).②35例患者临床表现包括发热100%(35/35),寒颤40.0%(14/35),贫血65.7%(23/35).脾大54.3%(19/35),脏器栓塞17.1%(6/35).③血培养阳性率为65.7%(23/35),其中13例为链球菌属.④35例患者中28例(80.0%)患者经单纯抗感染治疗后治愈,6例(17.1%)患者经手术联合药物抗感染治疗后治愈,有1例(2.9%)真菌感染患者死亡.⑤35例感染性心内膜炎患者中,无基础心脏病患者7例,有基础心脏病患者28例.7例无基础心脏病患者中有明确感染途径的占6例,28例有基础心脏病患者中有明确感染途径的占7例,两者感染途径阳性率比较差异有统计学意义(P=0.006).结论:感染性心内膜炎常见的基础心脏病是先天性心脏病、风湿性瓣膜病,二尖瓣脱垂;对于长期不明原因发热,应想到感染性心内膜炎的可能,尤其对于无基础心脏病、但有明确感染途径而长期发热者应高度怀疑感染性心内膜炎.超声心动图有助于感染性心内膜炎的诊断.     

Subacute bacterial endocarditis with positive cytoplasmic antineutrophil cytoplasmic antibodies and anti-proteinase 3 antibodies

OBJECTIVE: To report a potentially important limitation of antineutrophil cytoplasmic antibody (ANCA) testing: positive results in patients with subacute bacterial endocarditis (SBE). METHODS: We describe 3 patients with SBE who presented with features mimicking ANCA-associated vasculitis (AAV) and positive findings on tests for cytoplasmic ANCA (cANCA) by indirect immunofluorescence and for anti-proteinase 3 (anti-PR3)antibodies by antigen-specific enzyme-linked immunosorbent assay (ELISA). We also reviewed the published literature describing infectious diseases with (misinterpreted) positive ANCA results through a Medline search of English-language articles published between 1966 and January 1999. These previously reported cases were reinterpreted using an ANCA scoring system that combines the findings of immunofluorescence and antigen-specific ELISA testing. RESULTS: We are now aware of a total of 7 cases of SBE with positive cANCA and anti-PR3 antibodies. We are not aware of any cases of SBE associated with antimyeloperoxidase/perinuclear ANCA. Clinical manifestations mimicking AAV included glomerulonephritis, purpura, epistaxis, or sinus symptoms in 6 of the patients. Streptococcal species were identified in 5 patients, and cardiac valvular abnormalities were demonstrated in 6. All patients except 1, who died of a complication of SBE, recovered with antibiotic therapy. CONCLUSION: Findings of tests for anti-PR3/ cANCA antibodies may be positive in patients with SBE. When encountering ANCA positivity in patients suspected of having systemic vasculitis, physicians should take appropriate steps to rule out infectious diseases, including SBE, before committing the patient to long-term, aggressive immunosuppressive therapy.     

Anti-neutrophil cytoplasmic antibodies (ANCA) in malaria

Various autoantibodies like anti-nuclear antibodies (ANA), anti-double stranded DNA (anti-dsDNA), anti-histone antibodies (AHA), anti-neutrophil cytoplasmic antibodies (ANCA), anti-myeloperoxidase (anti-MPO), anti-proteinase3 (anti-PR3) and anti-lactoferrin (anti-LF) antibodies were studied in 173 acute hospitalised patients suffering from malaria of which 160 patients had P. falciparum and remaining 13 had P. vivax infection. Standard methods like indirect immunofluorescence (IIF) microscopy along with Confocal microscopy and ELISA were used for identifying and quantifying the autoantibodies and IIF patterns on PMN and HL-60 cells were studied for ANCA classification. Also HEp-2 cells were used for ANA detection, while estimation of anti-dsDNA, AHA, anti-MPO, anti-PR3 and anti-LF were tested using ELISA. Sera from malaria patients showed prominent immunofluorescence staining patterns where 23.8% cases had ANA in P. falciparum group as compared to 15.4% in P. vivax group and ANCA was found to be present in 20% in P. falciparum and 15.4% in P. vivax group. An interesting observation was that, of the total ANCA positives, 59% had p-ANCA, 5.9% had c-ANCA and 44.1% of the cases showed the 'atypical' or X-ANCA pattern. When p-ANCA positivity was compared with c-ANCA positivity among these patients, a good statistical correlation was noted with OR = 16, chi 2 = 16.43, EF = 0.46 and p-value = 5.037E 0.5. ELISA showed 31.2% anti-MPO and 6.2% anti-PR3 in P. falciparum cases while the two ANCA positive cases in P. vivax had anti-MPO. Anti-LF was found to be present in 40.6% cases. Neither the P. falciparum nor P. vivax contained autoantibodies with specificities similar to the characteristic lupus autoantibodies such as double stranded DNA (dsDNA). ANCA positivity develops in some types of malarial infection also with the presence of various autoantibodies which is important from a clinical point of view and should be carefully evaluated in those geographic areas where malaria is endemic. It also alerts us to the fact, whether in cases of repeated malarial infections in susceptible individuals, vasculitic disorders, which through ANCA pathways develop, could lead to renal and other complications.     

426例抗中性粒细胞胞浆抗体相关性小血管炎患者多系统临床表现和肾脏病理分析

目的 分析426例抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎患者多系统的临床和病理表现。方法 回顾性分析我院1997年-2004年6月检测并明确诊断的426例ANCA相关性小血管炎患者的临床病理资料。结果 426例患者中,70例胞浆型ANCA（cANCA）阳性,均识别蛋白酶3（PR3）;354例环核型ANCA（pANCA）阳性,均识别髓过氧化物酶（MPO）。201例（47．2％,201／426）患者是在发病后3个月内确诊。临床表现呈多器官受累,其中cANCA阳性者皮疹、关节痛、眼、鼻受累的发生率显著高于pANCA阳性者,而pANCA阳性者。肾脏受累和乏力的发生率显著高于cANCA阳性者。多数患者有贫血,血沉增快,C反应蛋白增高。采用糖皮质激素联合环磷酰胺进行强化免疫抑制治疗,诱导缓解期的缓解率为88．5％。结论 ANCA相关性小血管炎在我国并非少见,临床表现呈多器官受累,ANCA检测有助于早期诊断。     

The effect of anti-tuberculosis treatment on levels of anti-phospholipid and anti-neutrophil cytoplasmatic antibodies in patients with active tuberculosis

The aim of this study is to determine the prevalence and effect of anti-tuberculosis treatment on anti-phospholipid antibodies and anti-neutrophil cytoplasmatic antibodies (ANCA) in patients with active mycobacterial infections. Thirty-three consecutive patients (age 56 years, 26 males) with recently diagnosed active tuberculosis (TB) were enrolled. Data included clinical disease features, symptom duration, multidrug resistance and presence of HIV. Serum samples taken before and after TB treatment were frozen at ?20 °C and tested for anti-cardiolipin IgG (aCL), anti–β2 glycoprotein IgG (anti-β2GPI), anti-prothrombin, anti-proteinase 3 (PR3), myeloperoxidase (MPO), bactericidal/permeability (BPI) and lactoferrin. Thirty percent of patients had higher than cut-off value for anti-β2GPI, and 9 % had increased aCL. The levels of antibodies against β2GPI and aCL normalized post-treatment. A substantial proportion of patients had high baseline anti-PR3, MPO, BPI and lactoferrin levels. Most anti-lactoferrin and anti-MPO levels decreased post-treatment, while anti-PR3 increased in most of the baseline-positive patients. Some patients had de novo anti-PR3 and MPO formation after 6-month treatment. Patients with active TB have significantly increased anti-β2GPI and ANCA titers. While anti-β2GPI titers normalize post-treatment, ANCA behave in a complex way. Anti-TB treatment may induce normalization of anti-lactoferrin and anti-MPO, and de novo anti-PR3 and MPO formation.     

Low seroprevalence and poor specificity of antineutrophil cytoplasmic antibodies in tuberculosis

OBJECTIVES: Recently published findings suggested that antineutrophil cytoplasmic antibodies (ANCA), particularly those with a cytoplasmic (C-ANCA) labelling pattern and targeting proteinase 3 (anti-PR3), might be markers of tuberculosis (TB). This is a critical issue, because C-ANCA/anti-PR3 were considered to be a highly specific hallmark of Wegener's granulomatosis or microscopic polyangiitis and because TB may clinically mimic Wegener's granulomatosis. We therefore undertook a study with the aim of investigating further the prevalence and specificity of ANCA in TB. METHODS: We evaluated serum samples from 67 patients diagnosed with culture-proven TB and 10 previously untested control samples from patients known to be ANCA positive (four Wegener's granulomatosis and two microscopic polyangiitides) or negative. All 77 sera were screened for ANCA using commercially available indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA) for anti-PR3 and antimyeloperoxidase (MPO). IIF-positive and anti-PR3- and anti-MPO-negative sera were also tested for bactericidal/permeability-increasing protein, lactoferrin, elastase and cathepsin G specificities with commercially available ELISA. RESULTS: IIF detected ANCA in seven (10%) of the TB sera, including three C-ANCA and four atypical perinuclear-labelling ANCA. Only one IIF-negative specimen was anti-PR3 positive in ELISA. ANCA testing of the control sera yielded IIF and ELISA results concordant with previous findings, except for one borderline ELISA. CONCLUSION: Our results indicate that TB is associated with low ANCA seroprevalence and poor specificity, with no test serum showing combined C-ANCA/anti-PR3 activity. In a clinical setting of Wegener's granulomatosis/TB mimicry, such combined reactivity would seem to be more suggestive of Wegener's granulomatosis.     

以肥厚性硬脑膜炎为主要表现的肉芽肿性多血管炎临床病例分析

目的探讨肉芽肿性多血管炎(GPA)继发肥厚性硬脑膜炎(HCP)的临床特点。方法回顾性分析北京协和医院2004—2018年收治的GPA继发HCP病例资料的特点。结果①GPA患者315例,19例继发HCP,占6.0%;②男性12例,女性7例;年龄19~64岁,中位年龄57岁。③神经系统表现:19例均有头痛,16例颅神经受累。受累部位:额部8例,颞部8例,颅底8例(鞍旁4例,其中海绵窦3例,眶尖2例),小脑幕6例,大脑镰2例,顶部1例,枕部1例,1例合并硬脊膜炎。④系统表现:发热10例,体质量下降8例,肺部受累4例,肾脏受累3例,16例鼻窦炎,10例中耳炎,16例局限型GPA。⑤15例ANCA抗体阳性,8例蛋白酶3(PR3)-ANCA阳性,6例髓过氧化物酶(MPO)-ANCA阳性。⑥16例行腰椎穿刺检查:脑脊液压力9例升高、5例正常、2例降低;脑脊液蛋白升高10例。⑦15例(78.9%)伯明翰系统性血管炎评分(BVAS)>15分。⑧19例均使用糖皮质激素、免疫抑制剂治疗,其中12例行甲泼尼龙冲击治疗,12例鞘内注射地塞米松(或+甲氨蝶呤),19例病情均缓解。结论HCP是GPA少见且严重的表现,主要表现为颅高压和颅神经受累,多见于局限型GPA患者,常伴有全身疾病的活动,需积极治疗。     

The prevalence and target antigens of antithyroid drugs induced antineutrophil cytoplasmic antibodies (ANCA) in Chinese patients with hyperthyroidism

OBJECTIVE: Antithyroid drugs such as propylthiouracil (PTU) and methimazole (MMI) are common medications in Chinese patients with hyperthyroidism and PTU-induced antineutrophil cytoplasmic antibody (ANCA) positive vasculitis has been reported. The current cross-sectional study aimed to investigate the prevalence and the target antigens of ANCA in Chinese patients with hyperthyroidism pre- and post-antithyroid medication therapy. METHODS: Sera from 216 patients with hyperthyroidism in our hospital were collected from January to July in 2002. Patients were divided into four groups: untreated (n = 34); treated with PTU (n = 62); treated with MMI (n = 77); and treated with both PTU and MMI (n = 43). Indirect immunofluorescence (IIF) assay was used to detect ANCA and ANA. Antigen-specific ELISAs were used to detect antigen specificities. The known antigens included myeloperoxidase (MPO), proteinase 3 (PR3), human leukocyte elastase (HLE), lactoferrin, bactericidal/permeability-increasing protein (BPI), cathepsin G and azurocidin. RESULTS: 33/216 sera were IIF positive, 20 of the 33 samples were ANCA positive, 11 samples were ANA positive, and two samples were both P-ANCA and ANA positive. The prevalence of positive ANCA in patients receiving PTU (14/62, 22.6%) was significantly higher than that of untreated patients (1/34, 2.9%) and patients treated with MMI (0/77, 0), P < 0.017. Of the 22 IIF-ANCA positive samples, 12 (54.5%) sera recognized lactoferrin, seven (31.8%) sera recognized HLE, four sera recognized MPO and azurocidin respectively, three sera recognized PR3 and cathepsin G respectively, and one serum recognized BPI. Six of the 22 (27.3%) patients with ANCA positive had clinical evidence of vasculitis. All patients with MPO-ANCA and two of the three patients with PR3-ANCA had clinical vasculitis. CONCLUSION: PTU is associated with the production of ANCA in patients with hyperthyroidism. PTU-induced ANCA are due to polyclonal activation of B cells. Anti-MPO and anti-PR3 antibodies may associate the occurrence of clinical vasculitis.     

Anti-proteinase 3 antibodies in diffuse systemic sclerosis (SSc) with normotensive renal impairment: is it suggestive for an overlapping between SSc and idiopathic vasculitis?

OBJECTIVE: To test the prevalence of anti-neutrophil cytoplasmic antibodies (ANCA) in systemic sclerosis (SSc) and to verify a possible association of ANCA with normotensive renal involvement in SSc. PATIENTS AND METHODS: 51 patients affected by SSc, 35 with diffuse scleroderma (dSSc) and 16 with limited scleroderma (lSSc), were tested for ANCA by indirect immunofluorescence (IIF) on human ethanol and formalin-acetone-fixed granulocytes (before and after DNase treatment), by conventional enzyme linked immuno-sorbent assay (ELISA) and by capture-ELISA. RESULTS: Six out of 51 selected SSc patients had ANCA by IIF (11.7%) and five presented a perinuclear/nuclear atypical ANCA pattern. In all cases we only found anti-proteinase3 (aPR3) antibodies. All ANCA positive patients had diffuse form of SSc (17.1%), all were anti-Scl70 positive (aScl70), five patients had proteinuria, three had microscopic haematuria. All ANCA positive patients were normotensive with normal renin plasma levels, the mean erythrocyte sedimentation rate (ESR) was higher in this group compared to the other SSc patients. CONCLUSIONS: Our study shows that aPR3 is not rare in dSSc. According to the clinical and serological findings and to the recent literature, we can hypothesise that when ANCA are found in SSc, an overlapping of scleroderma with systemic necrotizing vasculitis should be suspected.     

Characteristics and Diagnostic Challenge of Antineutrophil Cytoplasmic Antibody Positive Infective Endocarditis

ObjectiveAntineutrophil cytoplasmic antibody (ANCA) has been reported in patients with infective endocarditis. Whether ANCA is associated with certain characteristics of infective endocarditis is unclear. The principal aim of this study is to investigate the clinical implications of ANCA in infective endocarditis and highlight the diagnostic challenge in ANCA-positive patients with infective endocarditis.MethodsA retrospective study was conducted in a tertiary hospital in China from August 2012 to December 2021. Patients with a diagnosis of infective endocarditis and available ANCA results were included in the study. The clinical and pathological characteristics were compared between ANCA-positive and ANCA-negative patients.ResultsA total of 237 patients were included. Forty three (18.1%) were ANCA-positive, predominantly c-ANCA/anti-PR3. Compared to ANCA-negative patients, ANCA-positive patients had longer disease duration (P = .004), more frequent purpura (P = .015), macrohematuria (P = .002), proteinuria (P = .043), acute kidney injury (P = .004), and rapidly progressive glomerulonephritis (P = .010). Histologic findings of 8 patients with infective endocarditis-associated glomerulonephritis were reviewed. Two ANCA-positive patients with infective endocarditis presented with pauci-immune necrotizing and crescentic glomerulonephritis. A total of 18.6% of ANCA-positive patients with infective endocarditis were misdiagnosed as ANCA-associated vasculitis.ConclusionsANCA is detected in a substantial proportion of patients with infective endocarditis. The presence of ANCA in infective endocarditis is associated with longer disease duration, more frequent purpura, and kidney involvement. ANCA-positive infective endocarditis may mimic ANCA-associated vasculitis, and the differential diagnosis is challenging. Whether ANCA is pathogenic in infective endocarditis-associated small vessel vasculitis requires further study.     

Laboratory Diagnosis of ANCA-Associated Vasculitis (AAV) Using a Combination of Immunofluorescence Test (IIFT) and Line Immunoassay (LIA): Single-Centre Report From India

IntroductionAnti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is a small vessel vasculitis with insufficient epidemiological estimates in India. We aimed to determine demographic, clinical features, and laboratory diagnosis of AAV patients presenting to a large tertiary care centre in India.Material and methods1289 patient samples were screened for ANCA by indirect immunofluorescence test (IIFT) and confirmation of ANCA target antigens was done by line immunoassay. Association between IIFT and LIA was determined in AAV.ResultsBy IIFT, ANCA was detected in 13.0% (168 out of 1289), of which 23.8% (40/168) were positive with P-ANCA pattern, 25.0% (42/168) were positive with C-ANCA and 47.6% (80/168) showed an atypical pattern. On evaluation with a line immunoassay, 6.7% (86/1289) were positive out of which 52.3% (45/86), 41.9% (36/86), 8.8% (6/86) were positive for anti-MPO, anti-PR3, and anti-GBM respectively. In eosinophilic granulomatosis with polyangiitis (EGPA) 87.5% (7/8), and microscopic polyangiitis (MPA/RLV) 91.3% (21/23), anti-MPO was the predominantly observed antibody. In granulomatosis with polyangiitis (GPA) anti-PR3 antibody was predominant in 87.5% (28/32) cases. Out of 168 IIF positive samples 8, 32, and 23 cases of EGPA, GPA, and MPA/RLV were observed respectively.ConclusionsThe primary aim of the study was to provide single-centre data to determine the laboratory diagnosis of AAV. A combination of IIFT and LIA was found to be an optimum testing strategy for the laboratory diagnosis of AAV.