变应性肉芽肿性血管炎

变应性肉芽肿性血管炎 ,是系统性血管炎变 ,以哮喘、坏死性血管炎、血管外肉芽肿、外周血嗜酸粒细胞增多和多器官组织嗜酸粒细胞浸润为特征。临床少见 ,根据组织病理确诊的病例统计 ,发生率约 2 4 / 10 0万人口。因首先由Ｃｈｕｒｇ和Ｓｔｒａｕｓｓ两位病理学家描述 ,通常又称Ｃｈｕｒｇ Ｓｔｒａｕｓｓ综合征 (Ｃｈｕｒｇ Ｓｔｒａｕｓｓｓｙｎｄｒｏｍｅ ,ＣＳＳ)1　病因和发病机制　　多数ＣＳＳ病因不明。有个案报道吸入变应原 ,如放线菌 ,以及使用别嘌呤醇、抗惊厥制剂等药物可诱发ＣＳＳ ,但极少见。自 1996年扎鲁斯特上市应用以来 …     

变应性肉芽肿性血管炎

变应性肉芽肿性血管炎是一种以哮喘、过敏性鼻炎、嗜酸粒细胞增多和全身性血管炎为特征的疾病。本文综述其流行病学、临床表现和诊断与治疗。并讨论其与抗白三烯药物的可能关系。     

变应性肉芽肿性血管炎

变应性肉芽肿性血管炎是一类病因不明、主要累及小和中等口径血管的系统性血管炎。于 195 1年首先由 Churg和Strauss描述 ,故又称 Churg- Strauss综合征 (CSS)。各民族、各地区均有报道 ,发病年龄 10～ 70岁不等 ,多数患者 2 0～ 40岁起病 ,男女患病率大致相等。1　病理改变CSS的病理特征为 :1嗜酸粒细胞浸润 ,血管周围结缔组织中可见正常嗜酸粒细胞 ,也可见浆颗粒消失、核固缩及碎裂的嗜酸粒细胞 ,伴胶原肿胀、坏死 ;2肉芽肿形成 ,巨细胞周围有大量嗜酸粒细胞围绕 ,外被上皮样细胞 ,多位于血管周围 ;3血管炎性坏死 ,表现为节段坏死、动…     

变应性肉芽肿性血管炎

1　病例报告　　患者男性,29岁,因夜尿增多2年,血压升高伴肾功能下降1个月,于1998年1月19日入院。缘于2年前无诱因出现夜尿增多,3～6次/夜,不伴其它症状,因此没有引起重视,亦未作任何检查。入院前1个月出现视物模糊伴头昏、乏力,测血压213/133ｋＰａ(160/100ｍｍＨｇ),尿蛋白3 ,ＲＢＣ0～1个/ＨＰ,ＢＵＮ138ｍｍｏｌ/Ｌ,ＳＣｒ456.3μｍｏｌ/Ｌ。给予心痛定、抑平舒降压,金水宝、保肾片等治疗,血压可降至正常,但肾功能仍未恢复,入院前1周血ＳＣｒ为357μｍｏｌ/Ｌ。病程中偶有脱发、眼干、口干,无浮肿、少尿,无发热、哮喘、鼻炎、皮疹、关节…     

变应性肉芽肿性血管炎

报道８例变应性肉芽肿性血管炎（Ｃｈｕｒｇ－Ｓｔｒａｕｓｓｖａｓｃｕｌｉｔｉｓ），该病临床主要以过敏性鼻炎（６９％）、副鼻窦炎（８８％）、哮喘（９８％～１００％）、多发性单神经炎（６６％～９８％）、肺内浸润、周围血嗜酸细胞显著增多为特征。病理学检查可见组织嗜酸细胞浸润，肉芽肿病变，坏死性血管炎。这些病理变化可局限或广泛分布，以上病变不一定同时出现，可孤立或混合存在。单次组织活检可能看不到病变全貌。按照１９９０年美国风湿病协会血管炎分会提出６项关于本病的诊断标准。本组８例中６例符合该诊断标准，２例由病理检查证实。对本病的诊断应侧重临床表现。本病对皮质激素反应良好，治疗效果较明显。     

变应性肉芽肿血管炎

变应性肉芽肿血管炎（CSS）临床病情以支气管哮喘（哮喘）为主,其归类属嗜酸粒细胞增多症范畴,又属中性粒细胞胞浆抗体（ANCA）相关性血管炎范畴,故类似的疾病和需鉴别的疾病较多而易于误诊,我科最近收治一例亦被误诊为哮喘达6年之久,为此临床医生认识此病甚为重要。     

变应性肉芽肿性血管炎伴脑梗死一例

变应性肉芽肿性血管炎(CSS)以哮喘、坏死性血管炎、血管外肉芽肿、外周血嗜酸性粒细胞增多和多器官组织嗜酸性粒细胞浸润为特征。临床上较少见，发病率约为2．4／100万。现将我们遇到的1例报告如下。     

变应性肉芽肿性血管炎25例临床分析

目的 探讨变应性肉芽肿性血管炎(Churg-Strauss综合征,CSS)的临床特点,以提高对本病的认识.方法 回顾性分析2001年1月至2010年11月北京协和医院收治的25例变应性肉芽肿性血管炎患者的临床资料,包括临床表现、实验室资料、辅助检查及病理等.结果 男15例,女10例,发病年龄18～ 72岁,平均(45±16)岁.病变累及全身多个系统及器官,如呼吸系统、神经系统、皮肤、消化系统、心脏、肾脏及关节肌肉等,以支气管哮喘(简称哮喘)为首发症状的占80.0％( 20/25),病程中出现哮喘的占96.0％( 24/25),鼻窦炎多表现为多组鼻窦受累,以上颌窦为主,占61.1％ (11/18);神经系统受累多表现为外周神经的受累,占72.7％ (16/22);中枢神经系统受累多表现为脑血管事件.皮肤受累占60.0％ (15/25),心脏受累占48.0％( 12/25),肾脏受累占48.0％(12/25),消化系统受累占32.0％( 8/25).实验室检查中,IgE阳性率为90.0％,抗中性粒细胞抗体阳性率为32.0％,以核周型抗中性粒细胞胞质抗体为主.CSS的肺部CT多表现为斑片状浸润,占71.4％( 15/21),双侧多见;肌电图多表现为多发性单神经炎,以腓神经及腘神经受累多见.心电图多无异常发现,但超声心动图70.0％ (14/20)有异常.病理主要表现为嗜酸粒细胞浸润,血管炎及血管外肉芽肿.结论 CSS的临床表现复杂多样,但均缺乏特异性,综合临床表现、辅助检查及病理表现方可完成诊断,及时诊断,早期治疗对患者的预后有帮助.     

变应性肉芽肿性血管炎误诊2例分析

目的总结变应性肉芽肿性血管炎(Churg-Strauss syndrome,CSS)的诊治经验及误诊原因。方法回顾性分析近年来本院收治诊断的2例被误诊的CSS患者的临床表现、发病机制、影像学表现、诊断和治疗的特点。结果 2例患者中1例被误诊为难治性哮喘,1例被误诊肺结核,提示CSS患者的临床表现早期可能与相关疾病很多相似之处,容易漏诊、误诊而延误诊治。结论临床医师对本病的认识不足是误诊的主要原因,提高对本病的认识程度,熟悉其病理生理,临床特点,影像学特征是减少误诊的关键。     

变应性肉芽肿性血管炎相关性哮喘二例

例1 男，74岁，因反复喘息伴咳嗽29年，症状加重伴发热2周，于2001年3月24日入我院。患者息1972年起反复出现喘息和咳嗽，受凉或接触敌敌畏等可诱发，一直按支气管哮喘（简称哮喘）或肺炎治疗，给予抗感染、糖皮质激素（简称激素）和氨茶碱等治疗可缓解。     

Syndrome de Churg-Strauss

Churg–Strauss syndrome (CSS) is a granulomatous, necrotizing vasculitis affecting small-sized blood vessels. The disease is characterized by asthma, eosinophilia and extra-respiratory manifestations. ANCA are found in one-third to half of the patients with CSS. The prognosis depends on whether there is cardiac and/or gastrointestinal involvement. Steroids are indicated in every case; in severely affected cases, an immunosuppressive agent, e.g. cyclophosphamide, should be prescribed as well. Nevertheless, the prognosis of CSS remains very good; the remission rate approaches 90%.     

Churg-Strauss Syndrome with pleural involvement

A 51-year-old Japanese man with Churg-Strauss Syndrome (CSS) diagnosed by pleural biopsy is described. He was hospitalized because of high fever and bilateral knee, elbow and shoulder joint pain. Chest roentgenogram and chest computed tomography (CT) scan revealed bilateral massive pleural effusion. Pleural biopsy revealed eosinophilic infiltration and necrotizing granulomas. He was treated with oral prednisolone and his symptoms improved. This is the first report of CSS diagnosed by pleural biopsy.     

Churg-Strauss Syndrome: An Update

Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody-associated vasculitis. The presence of asthma, usually of adult onset, along with other allergic symptoms, peripheral and tissue eosinophilia, is specific to this disease. These features usually help clinicians distinguish it from other types of small vessel vasculitis and should alert clinicians about its presence. Two different clinical subtypes defined by the presence of antineutrophil cytoplasmic antibodies recently have been recognized. Recent advances in the treatment and pathophysiology of Churg-Strauss syndrome are reviewed in this article.     

Churg-Strauss Syndrome Associated with Montelukast Therapy

Churg-Strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. Several cases of eosinophilic conditions including Churg-Strauss syndrome have recently been reported in asthmatic patients being treated with antileukotriene receptor antagonists. However, whether these drugs have a direct pathogenic role remains controversial. We describe two patients who developed Churg-Strauss syndrome after starting treatment with montelukast.     

Churg-Strauss综合征发病机制研究进展

Churg-Strauss综合征(CSS)是一种常伴有哮喘的系统性血管炎.迄今为止,CSS的病因和发病机制仍不甚明了.外周血和组织中嗜酸性粒细胞(EOS)增多及EOS代谢产物水平升高,表明EOS参与CSS发病.此外,T细胞分泌细胞因子和致炎因子大量释放,提示T细胞激活."抗中性粒细胞胞浆抗体(ANCA)-细胞因子-序列理论"认为CSS的发病与ANCA和中性粒细胞也有关.     

A Case with Multiple Colonic Ulcers Simulating Churg-Strauss Syndrome

Abstract: Churg-Strauss syndrome (CSS), a relatively rare disorder which is associated with serious complications, has a highly variable course and several possible manifestations. We present the case of a 35-year-old woman with a history of bronchial asthma, admitted for evaluation of lower abdominal pain and melena, whose clinical course had certain features in common with CSS. On admission, the white blood cell count was 45,300/mm³ with 65% eosinophils, and the serum immunoglobulin E (5,300 u/ml) level was remarkably elevated. At colonoscopy, there were shallow ulcers, irregular in shape, throughout the large intestine. Abdominal pain and melena were relieved by oral administration of prednisolone. Most previously reported cases have not been recognized as having colonic involvement until surgery or autopsy. In only a few reports of CSS and related disorders were colonoscopic examination findings described.