变应性肉芽肿性血管炎研究新进展

变应性肉芽肿性血管炎是一种以支气管哮喘(简称哮喘)、血管外坏死性肉芽肿、外周血嗜酸粒细胞增多和组织嗜酸粒细胞浸润为特征的系统性小血管炎,1951年由Churg和Strauss首先报道,故称之为Churg-Strauss综合征,即CSS.     

Churg-Strauss综合征

Churg-Strauss综合征(Churg-Strauss syndrome,CSS)是一种血管炎性疾病,表现为全身小至中等血管坏死性血管炎、血管内外肉芽肿形成、外周血嗜酸粒细胞增多以及组织及血管周围嗜酸粒细胞浸润。该病又称为变应性肉芽肿血管炎,但国内外文献多以CSS描述本病。CSS发病率为0.5/10万~6     

重症Churg-Strauss综合征一例报告

Churg-Strauss综合征（Churg-Strauss syndrome,CSS）是指伴有支气管哮喘和外周血嗜酸性粒细胞增多,累及呼吸道,有大量嗜酸性粒细胞浸润和血管外肉芽肿形成的,影响小到中等大小血管的坏死性血管炎。本病临床上较为罕见,本文报道一例重症CSS的诊治经过以提高临床上对此病的认识。     

老年变应性肉芽肿性血管炎1例诊治体会

变应性肉芽肿性血管炎,又称Churg-Strauss综合征(CSS).是一种以哮喘、血和组织中嗜酸粒细胞增多、血管外坏死性肉芽肿为特征的系统性小血管炎.该病临床少见,国外报道其发病率约2.2～6.8/百万~([1]),临床上易被漏诊.本文通过总结1例老年CSS患者的诊治体会,以期提高临床医师对该病的认识.     

Churg-Strauss综合征

作者综述一种与结节性多动脉炎不同的疾病,其主要表现为哮喘、发热、嗜酸细胞增多及多系统血管炎,命名为 Churg-Strauss 综合征。本病病因尚表明瞭,病理学改变的主要特征是嗜酸细胞组织浸润、血管肉芽肿形成及坏死性血管炎。本文对其临床分期及表现、诊断、治疗等进行了较为详细的描述。     

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.     

哮喘难以控制需警惕变应性肉芽肿性血管炎

变应性肉芽肿性血管炎（allergic granulomatous angiitis，AGA）是一种以哮喘、血和组织中嗜酸粒细胞增多、血管外坏死性肉芽肿为特征的系统性小血管炎”。1951年由Churg和Strauss首先描述，故又称Churg-Strauss综合征（Churg-StraussSyn-drome，CSS）”。本病临床少见，发病率约为2．4／百万，在哮喘病人中约64．4／百万。由于本病患者在出现嗜酸粒细胞增多和系统性血管炎症状前常有数年难治性哮喘（difficult-to-controlasthma）的病史，因而很容易误诊。本文结合我们最近诊治的一个病例，谈谈对CSS诊治的体会。     

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。     

嗜酸性粒细胞增多症患者心脏受累的临床和病理表现

目的 探讨嗜酸性粒细胞增多症心脏受累的临床和病理特点.方法 分析7例嗜酸性粒细胞增多症累及心脏且行心内膜活检或尸检患者的临床和心肌病理资料.结果 7例患者中5例为男性,平均年龄51岁;特发性嗜酸性粒细胞增多综合征4例,Churg-Strauss综合征3例.7例患者外周血嗜酸性粒细胞均有明显升高.心脏受累的表现有心绞痛、心肌梗死、心肌病变合并心功能不全、瓣膜病变合并严重心功能不全、房室传导阻滞和猝死.病理显示有嗜酸性粒细胞浸润、肉芽肿形成、坏死性血管炎、心肌细胞坏死、间质纤维化和心内膜纤维化.经糖皮质激素和免疫抑制剂治疗后大部分患者外周血嗜酸性粒细胞降低或正常,心脏病变稳定甚至好转.结论 嗜酸性粒细胞增多症伴发心脏受累的临床表现多样而且预后差;早期识别并积极治疗有助于改善患者的预后.     

变应性肉芽肿性血管炎26例临床分析

目的分析变应性肉芽肿性血管炎(CSS)的临床特点,以提高诊治水平。方法回顾性分析1997年1月至2005年6月北京协和医院及天津市第一中心医院收治的26例CSS患者的临床资料(既往病史、临床表现、实验室检查、组织病理检查及治疗情况)。结果外周血嗜酸性粒细胞增多者占96·2%,哮喘发生率为84·6%,皮肤损害73·1%,肺浸润61·5%,多发单神经炎57·7%,消化系统受累46·2%,心血管系统损害42·3%,肾脏损害29·4%。病理检查血管外肉芽肿不多见。25例应用激素和免疫抑制剂治疗后好转。结论变应性肉芽肿性血管炎临床表现复杂,激素治疗反应好。有哮喘或嗜酸性粒细胞增多者需考虑CSS的可能,必要时行多系统多部位活检。     

Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases.

Churg-Strauss syndrome is an eosinophil-associated, small vessel granulomatous vasculitis, characterized by late onset asthma, upper airways disease, eosinophilia, and clinical manifestations of systemic vasculitis. Several cases of Churg-Strauss syndrome have been recognized in patients treated with cysteinyl leukotriene-receptor antagonists and weaned off systemic corticosteroids. These cases have led to a general warning on the possible development of Churg-Strauss syndrome after taking cysteinyl leukotriene-receptor antagonists. The authors report five cases of Churg-Strauss syndrome in severe steroid dependent asthmatics in whom inhaled corticosteroids allowed systemic corticosteroid withdrawal. It is concluded that physicians should monitor patients carefully when severe asthma is controlled with any substance allowing withdrawal from (or even avoidance) of systemic corticosteroids. Case-control studies should identify more precisely the risk factors of Churg-Strauss syndrome.     

Das Churg-Strauss-Syndrom

Churg-Strauss syndrome is a systemic ANCA-associated vasculitis arising almost exclusively in patients with a pre-existent asthma. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. The morphological substrate is an eosinophilic necrotizing vasculitis. Other eosinophilic disorders such as parasitic diseases, allergies and idiopathic hyper-eosinophilic syndrome have to be excluded. The mainstay of therapy is high-dose corticosteroids with the addition of cytotoxic drugs in patients with poor prognosis.     

Churg-Strauss syndrome

Churg-Strauss syndrome is a systemic ANCA-associated vasculitis arising almost exclusively in patients with a pre-existent asthma. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. The morphological substrate is an eosinophilic necrotizing vasculitis.Other eosinophilic disorders such as parasitic diseases, allergies and idiopathic hyper-eosinophilic syndrome have to be excluded. The mainstay of therapy is high-dose corticosteroids with the addition of cytotoxic drugs in patients with poor prognosis.     

全身小血管炎合并严重心肌受累10例分析

目的 通过对小血管炎引起的严重心肌受累病例的分析提高对本病的认识.方法 总结北京协和医院近12年共10例小血管炎心肌受累病例临床资料及预后.结果 小血管炎心肌受累主要表现心肌收缩功能受损,心脏超声检查可早期诊断并监测病情,糖皮质激素治疗可改善心功能.结论 小血管炎心肌受累仍属罕见病,早期诊断、及时治疗可以改善症状和预后.     

The status of Churg-Strauss syndrome among other hypereosinophilic, granulomatous and vasculitic diseases

The Churg-Strauss syndrome is a disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Only few patients are identified as having this syndrome. The three histological criteria are necrotizing vasculitis, tissue infiltration by eosinophils, and extravascular granulomas; they often do not coexist in one patient. To find a clinical approach to diagnosis, it is necessary to exclude other disorders with hypereosinophilia, granulomas, and vasculitis. In regard to this clinical viewpoint it seems that the syndrome is not as rare as may be assumed according to the relevant autopsy findings. Two cases are reported in which the Churg-Strauss syndrome developed together with rheumatoid arthritis; in one case it was likely triggered by treatment with D-penicillamin.     

Asthma bronchiale und Churg-Strauss-Syndrom

Churg-Strauss syndrome (CSS), which is synonymous with eosinophilic granulomatosis with polyangiitis, is characterized by mostly severe bronchial asthma, eosinophilia and systemic vascular inflammation which can have many different forms of organ manifestation. It represents an important therapy-relevant differential diagnosis of severe asthma. Typical features are a course which runs in phases with a prodromal phase, a phase of eosinophilic tissue infiltration and a vasculitis phase. Depending on the clinical presentation and detection of antineutrophil cytoplasm antibodies (ANCA) two phenotypes can be distinguished. Bronchial asthma in CSS is treated according to the standards for asthma therapy and in the vasculitis phase a combined therapy with systemic corticosteroids and immunosuppressants is often necessary depending on the severity of the disease and organ manifestations. Current data suggest positive effects of biologicals. A review of the pathogenesis, clinical features, diagnostics and therapy of CSS is given based on a current literature search.     

A case of Churg-Strauss syndrome in which positive rheumatoid factor and MPO-ANCA preceded the development of clinical symptoms of vasculitis]

A 52-year-old man in whom bronchial asthma had been diagnosed in 1995 was admitted for the treatment of Churg-Strauss syndrome in June 1997. Positive tests MPO-ANCA and rheumatoid factor preceded the symptoms of vasculitis for several months. A skin biopsy revealed infiltration of eosinophils in the vessel walls, and the diagnosis of Churg-Strauss syndrome was confirmed. After systemic administration of corticosteroids, the symptoms other than mononeuritis improved markedly, and his MPO-ANCA and rheumatoid factor became negative. Rheumatoid factor and MPO-ANCA may be useful for the early diagnosis of Churg-Strauss syndrome in patients with bronchial asthma in which a well-controlled disease develops into an intractable condition.     

Delayed relapse of Churg-Strauss syndrome manifesting as colon ulcers with mucosal granulomas: 3 cases

Churg-Strauss syndrome (CSS) is characterized by small vessel vasculitis and extravascular granulomas. The American College of Rheumatology classification criteria for CSS include asthma, eosinophili, and clinical manifestation of vasculitis. Gastrointestinal (GI) manifestations occur in 30% of patients, but are inaugural in only 16%. They denote vasculitis of the stomach and small bowel wall, and consist in protean, nonspecific pain. GI involvement is of adverse prognostic significance in CSS. Ulcer formation in the GI tract mucosa is a rarer manifestation, usually discovered upon laparotomy or autopsy. We describe 3 new cases of colonic ulcers in CSS. Unusual features were diagnosis of the ulcers during a delayed relapse and presence of eosinophilic granulomas within the mucosa.     

Multiple tracheobronchial mucosal lesions in two cases of Churg-Strauss syndrome

Churg-Strauss syndrome (CSS) is characterized by hypereosinophilia and a systemic necrotizing vasculitis seen almost exclusively in patients with asthma. The most common pathological findings in the chest in CSS are eosinophilic pneumonia, necrotizing vasculitis and granulomatous inflammation (extravascular granuloma). However, tracheobronchial mucosal lesions have rarely been reported in CSS. The authors report two patients with CSS who had multiple tracheobronchial mucosal lesions that were found by fibreoptic bronchoscopy. They were tiny nodular lesions and necrotizing bronchial inflammation with many eosinophils was observed upon pathological examination. The authors concluded that tracheobronchial mucosal lesions may be one of the manifestations of vasculitis seen in CSS.     

Allergic angiitis with granulomatosis: Churg-Strauss syndrome. Retrospective study of 16 cases

Sixteen patients with Churg-Strauss syndrome (CSS), a disorder characterized by hypereosinophilia and systemic vasculities which complicate preexisting asthma, were analyzed. The mean duration of asthma before CSS was 8 years; peripheral blood eosinophilia was always greater than 1,900/microliters and exceeded 5,000/microliters in 14 cases. The clinical manifestations were the following: 16 in generally poor condition with fever; 12 peripheral neuropathies; 11 cutaneous lesions; 9 pericardial or myocardial involvement; 9 digestive disorders; 9 muscular or articular diseases; 5 renal involvement, all associated with the vasculitis; 7 upper respiratory tract disorders. Chest radiographs showed pleuropulmonary or cardiac anomalies in 11 patients. The diagnosis was confirmed histologically in 11 cases, however, no clinical, biological or evolutive differences were observed between these patients and those with negative biopsies (5). Follow-up for 6.35 +/- 5.55 years was characterized by relapses always preceded by increased eosinophilia. Fourteen patients were successfully treated with corticosteroids, associated with cyclophosphamide in 7 of them. Five-year survival was 87%. Four deaths occurred, all CSS-associated, two because of a poorly adapted therapeutic regimen. The need for rapid and effective treatment must be stressed. The diagnosis can be made based on clinical manifestations alone before histological confirmation can be obtained.