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.     

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 associated with leukotriene receptor antagonists (LTRA)

Churg–Strauss syndrome (CSS) is a rare vasculitic disorder that generally occurs in patients with bronchial asthma. CSS is being increasingly recognized in asthmatic patients treated with leukotriene receptor antagonists. However, the nature of this relationship remains to be elucidated. The present report describes three asthmatic patients who developed clinical manifestations highly suggestive of CSS, although one patient lacked the presence of eosinophilia. The patient, however, exhibited biopsy-proven cutaneous necrotizing vasculitis, which improved after withdrawal of montelukast. The second patient presented with systemic constitutional signs including fever, malaise, arthralgias, clinical jaundice, peripheral blood eosinophilia, and biopsy-proven eosinophilic hepatitis. The third patient also had circulating eosinophilia, scleritis, and arthritis. All patients improved after discontinuation of the leukotriene receptor antagonist (montelukast).     

A retrospective study of bronchial hyperresponsiveness in patients with asthma before the onset of Churg-Strauss syndrome.

Asthma is one of the most common clinical symptoms in Churg-Strauss syndrome (CSS). However, it is not known how lung function and bronchial hyperresponsiveness (BHR) prior to the development of CSS differs from asthmatics do not develop CSS. This retrospective cohort study was conducted to predict the onset of CSS and facilitate diagnosis in the early phase of the disease. We examined 24 pre-CSS asthmatic patients and 294 non-CSS asthmatic patients for clinical features, percent forced expiratory volume at 1 second (%FEV1), BHR to acetylcholine, and evaluated eosinophils (%) in the peripheral blood at their first hospital visit for asthma treatment. All of the 24 pre-CSS patients had adult-onset asthma. The asthma of 87.5% of pre-CSS patients at the first hospital visit before the onset of CSS was severe and was complicated by sinusitis. The eosinophils (%) in the peripheral blood was significantly higher than in non-CSS asthmatic patients. The %FEV1 in both the patients with severe asthma and the patients who developed CSS was lower than in patients with mild or moderate asthma. However, BHR in pre-CSS patients was significantly better than in non-CSS patients with severe asthma and was as mild as in patients with mild asthma. Patients who developed CSS had clinically severe asthma before the onset of CSS. The severity of their asthma was related to airflow limitation and eosinophilic inflammation in the peripheral blood, but not to BHR. These findings should prove useful in future early diagnosis and treatment of CSS.     

Churg-Strauss综合征并发急性冠状动脉综合征的荟萃分析

目的分析Churg-Strauss综合征（CSS）并发急性冠状动脉综合征（ACS）患者的临床特点、治疗和结局。方法对北京协和医院就诊的3例及相关英文文献报道的8例CSS并发ACS患者的临床特点、冠状动脉造影（CAG）所见和治疗反应进行分析。结果本研究及文献报道的CSS并发ACS患者共11例,其中本研究3例、文献报道8例,男女比为4：7。11例ACS均出现在CSS发病初期,其中心肌梗死9例、心绞痛8例;CAG显示70%（7/10）为痉挛性多支血管狭窄或闭塞,动脉瘤1例。心脏外表现,包括支气管哮喘（11/11）、副鼻窦炎（7/11）、周围神经炎（5/11）、嗜酸性肺炎（4/11）及胃肠道受累（3/11）等。血清学特点为显著高嗜酸性粒细胞血症[（1.2～29.6）×109/L],抗中粒细胞胞浆抗体均阴性。CSS并发的ACS对常规冠心病二级预防治疗反应差,大剂量皮质激素和环磷酰胺联合治疗后病情稳定。结论 CSS并发的ACS以嗜酸性粒细胞相关的冠状动脉痉挛导致的多支血管严重狭窄或闭塞为特点,常规冠心病二级预防治疗反应差,联合应用大剂量皮质激素与环磷酰胺治疗可显著改善病情。     

Churg-Strauss Vasculitis. Description of 9 cases

IntroductionChurg-Strauss Síndrome (SCS) is a necrotizing vasculitis affecting small to medium-sized vessels, characterized by lung involvement, asthma and peripheral blood eosinophilia, and pathologically by the presence of granulomas and eosinophilic infiltrates.ObjectivesThis report analizes the characteristics of 9 patients with SCS diagnosed in an university referral center.Patients and methodsRetrospective study. Between 1984 and 2007 nine patients with SCS were diagnosed in our center. Epidemiological, clinical, laboratory test as well as pathologic studies and treatment required were retrospectively analyzed.ResultsNine patients (7 males). The mean age at the time of diagnosis was 51 years (range 23–76 years). Eight of these patients had history of asthma. The more frequent organs involved were the skin (66%), musculoesqueletical system (66%), peripheral nervous system (55%) and the lung (55%). All patients presented peripheral eosinophilia. ANCA positivity was demonstrated in 6 patients (66%), most of the patients with the p-ANCA pattern. All patients were treated with corticosteroids, and in 8 immunosupressant treatment was required, mainly cyclophosphamide.ConclusionsIn this report, 9 patients with SCS are presented. Clinical characteristics are similar with the observed in other reports. We observed a major positivity of ANCA. Most of the patients were treated with corticosteroids and inmunosupresants, but the treatment should be tailored depending on the involvement of the patient.     

Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature

A rare case of combined Churg–Strauss syndrome (CSS) and allergic bronchopulmonary aspergillosis (ABPA) was presented. A 41‐year‐old woman was diagnosed with CSS based upon asthma, eosinophilia (23%), chest radiographic findings, paranasal sinusitis, peripheral neuropathy and positive p‐ anti‐neutrophil cytoplasmic antibodies (pANCA). The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The clinical investigation features were reviewed in the patients with combined CSS and ABPA. All patients had the time sequence of the development of CSS after ABPA uniformly, suggesting immunopathogenesis involving the emergence of CSS. The role of lung biopsy in the diagnosis of the condition was emphasized. Please cite this paper as: Ren S. Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature. Clin Respir J 2013; 7: e6–e10.     

Reevaluation of eosinophilic pneumonia and its diagnostic criteria.

Eosinophilic pneumonia has been defined as pulmonary infiltration of the lung by eosinophils that may or may not be accompanied by an excess of these cells in the peripheral blood. However, the concept of this disease and its nomenclature have not yet been established. In the present study, the clinical course of 11 cases of eosinophilic pneumonia, which were clinico-pathohistologically diagnosed and found not to be associated with organic disorders producing peripheral blood eosinophilia, were investigated extensively and compared with various types of eosinophilic pneumonia, as previously reported. Of five cases of acute eosinophilic pneumonia with a history (less than 1 month) of symptoms before diagnosis, a short clinical course, and no recurrence, four cases showed peripheral blood eosinophilia, and four cases did not require treatment with steroids. Of six cases of chronic eosinophilic pneumonia with a history (greater than 2 months) of symptoms before diagnosis, a prolonged clinical course, and recurrence, all showed peripheral blood eosinophilia, four cases required treatment with steroids, and four cases were recurrent. In one case with simultaneous occurrence of asthma and another case with asthma that occurred 4 years after the appearance of eosinophilic pneumonia, abnormal shadows on chest roentgenograms had continued for several years. These results suggested that various types of previously reported eosinophilic pneumonia classified by sex, the presence or absence of peripheral blood eosinophilia, the degree of clinical symptoms or peripheral blood eosinophilia, and the degree of abnormalities on the chest roentgenograms should be extensively reevaluated.     

The lung in systemic vasculitis

Pulmonary vasculitis is usually caused by one of three disorders: (1) Wegener's granulomatosis (WG); (2) Churg-Strauss syndrome (CSS), or allergic angiitis and granulomatosis; or (3) a nonspecific small vessel systemic necrotizing vasculitis (SNV), or microscopic polyarteritis. WG, the most common cause of lung vasculitis, has features of a granulomatous vasculitis of the upper airway and lung and widespread small vessel vasculitis involving the kidneys and other organs. The features of pulmonary WG overlap with those of malignancy and infectious granulomatous lung disease; accurate diagnosis generally requires open lung biopsy. CSS is defined by the triad of asthma, eosinophilia, and systemic vasculitis. Easily accessible tissues should be biopsied, but the clinical features are so distinctive that tissue biopsy is not invariably required for diagnosis. CSS must be differentiated from other diseases that cause pulmonary infiltrates with eosinophilia, including infections. Nonspecific SNV causes diffuse alveolar hemorrhage due to pulmonary capillaritis. Concomitant segmental necrotizing glomerulonephritis is almost always present. Diagnosis is made by renal biopsy, compatible extrarenal features, exclusion of nonimmune causes of lung hemorrhage, and exclusion of WG to the extent possible.     

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.     

Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings

STUDY OBJECTIVES: To describe the radiologic and clinical findings of Churg-Strauss syndrome (CSS) and its thoracic manifestations. DESIGN: We used retrospective analysis to review and characterize the radiographic, thin-section CT, and clinical findings of CSS. PATIENTS: The study involved nine patients with CSS. The patients included four men and five women, whose ages ranged from 18 to 60 years (median, 35 years). Thin-section CT scans and chest radiographs were retrospectively analyzed by three radiologists in consensus. Clinical data were obtained by chart review. Histologic samples were available in eight patients. RESULTS: All patients had a history of asthma averaging 28 months (range, 4 to 72 months) prior to the initial symptom of vasculitis and marked peripheral blood eosinophilia (mean peak count, 8,726/microL; range, 3,000 to 32,000/microL; mean differential count, 41%; range, 19 to 67%). All patients had systemic vasculitis involving the lung and two to four extrapulmonary organs, most commonly the nervous system (n = 8) and skin (n = 7). Chest radiographs showed bilateral nonsegmental consolidation (n = 5), reticulonodular opacities (n = 3), bronchial wall thickening (n = 3), and multiple nodules (n = 1). The most common thin-section CT findings included bilateral ground-glass opacity (n = 9); airspace consolidation (n = 5), predominantly subpleural and surrounded by the ground-glass opacity; centrilobular nodules mostly within the ground-glass opacity (n = 8); bronchial wall thickening (n = 7); and increased vessel caliber (n = 5). Other findings were hyperinflation (n = 4), larger nodules (n = 4), interlobular septal thickening (n = 2), hilar or mediastinal lymph node enlargement (n = 4), pleural effusion (n = 2), and pericardial effusion (n = 2). CONCLUSIONS: In CSS, thoracic organs are invariably involved with additional diverse manifestations. The possibility of CSS should be raised in patients with a history of asthma and hypereosinophilia who present with thin-section CT findings of bilateral subpleural consolidation with lobular distribution, centrilobular nodules (especially within the ground-glass opacity) or multiple nodules, especially in association with bronchial wall thickening.     

Biliary tract disease: a rare manifestation of eosinophilic gastroenteritis

Eosinophilic gastroenteritis (EGE) is a rare inflammatory disease characterized by diffuse or scattered eosinophilic infiltration of the digestive tract and usually by peripheral blood eosinophilia. The most common presenting symptoms of EGE are abdominal pain, vomiting and diarrhea, but clinical features depend on which layers or location of gastrointestinal tract are involved. Treatment with corticosteroids results in clinical and histological remission in most patients and surgery can be avoided if a correct diagnosis is made. Previous history of allergy is a key to diagnosing EGE, but peripheral eosinophilia may be absent in some patients under concomitant treatment with corticosteroids. Radiological and endoscopic findings are also nonspecific and diagnosis must always be histologically confirmed. The gastrointestinal involvement is patchy in distribution, so more than one panendoscopic examination is often necessary to establish the diagnosis, and surgical or CT-guided full-thickness biopsy is needed in patients with muscular or serosal involvement. It emphasises the importance of a high index of clinical suspicion, which mainly depends on knowledge of natural history of the disease. We report here a case of EGE associated with transmural eosinophilic cholecystocholangitis, in a patient who presented with dyspeptic symptoms and recurrent cholestasis responsive to corticoesteroids. To our knowledge, this patient represents the second case, in the English literature, in which corticoid-responsive cholangitis was associated to histologically proven eosinophilic cholecystitis and gastrointestinal involvement, suggesting that EGE must always be considered in the differential diagnosis of biliary tract disease in patients with eosinophilia and/or atopic diseases.     

Churg‐Strauss syndrome presenting as acute abdomen: are gastrointestinal manifestations an indicator of poor prognosis?

Churg Strauss syndrome (CSS) is a rare syndrome of vasculitis of the small to medium vessels, characterized by a triad of asthma, eosinophilia and atopic sinusitis. Although asthma is the most common manifestation, it is rarely associated with poor prognosis. Severe gastrointestinal tract manifestations are associated with worse outcomes. Patients with CSS at initial presentation, like our patient, may not meet all four American College of Rheumatology criteria to establish a diagnosis; however, clinicians should have a high index of suspicion to recognize the disease early in its course and treat aggressively, thereby decreasing the mortality and morbidity associated with this disease.     

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.     

Bronchial obstruction and the ideopathic hyper-eosinophilic syndrome

The idiopathic hyper-eosinophilic syndrome is defined as a peripheral blood eosinophilia greater than 1.5 x 10(9)/litre present for at least 6 months and associated with visceral involvement. It may only be accepted after carefully excluding the other common or rare causes of hyper-eosinophilia and should remain a diagnosis of exclusion. The associated visceral lesions are multiple and non-specific. Among these neurological, digestive, dermatological and cardiac manifestations are the most frequently described. Whereas pleuro-pulmonary involvement is also common, asthma is rarely reported. We report a case of ideopathic hyper-eosinophilic syndrome presenting as asthma. This was secondary to eosinophilic infiltration of the bronchial mucosa as demonstrated by cytological examination of induced sputum.     

Factors related to prognosis and relapse in acute myelomonocytic and monocytic leukemias (M4 and M5)

To analyse the factors which were related to prognosis at first examination and early diagnosis of relapse in complete remission phase, 26 patients with acute myelomonocytic leukemia (M4) and acute monocytic leukemia (M5) were investigated. There was a tight relationship between age and remission rate in patients with M4 and M5. Six of M4 with eosinophilia (M4Eo) patients revealed 83.3% as remission rate with good prognosis in the survival curve. LDH level of them was lower than other patients significantly. In order to diagnose relapse before clinical manifestations, it was useful to follow up number of mature monocytes (over 600/microliters) in the peripheral blood.     

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??Abstract??Pulmonary Infiltrates with eosinophilia??clinically referred to as eosinophilic lung diseases (ELD)??are a heterogeneous group of disorders characterized by pulmonary infiltration associated with alveolar eosinophilia with or without peripheral eosinophilia.They are classified generally as primary ELD??secondary ELD??and other diseases occasionally associated with eosinophils.Clinical manifestation of these disorders can be varied??and their presentation can be mild or fatal.Accurate diagnosis is based on a full history and examination??along with clinical manifestations??radiological imaging and laboratory findings.Corticosteroids are the mainstay of treatment for ELD and most patients will require prolonged courses of daily steroids.The dose and use duration of corticosteroids should be individualized.     

Churg-Strauss syndrome presenting with acute myocarditis and cardiogenic shock

Churg-Strauss syndrome (CSS) is a multisystem disorder characterised by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. We report the case of a 22 year-old man admitted to the intensive care unit for acute myocarditis complicated with cardiogenic shock. Eosinophilia, history of asthma, lung infiltrates, paranasal sinusitis, glomerulonephritis, and abdominal pain suggested the diagnosis of CSS. Cardiac MRI confirmed cardiac involvement with a diffuse subendocardial delayed enhancement of the left ventricular wall, and a left ventricular ejection fraction (LVEF) of 30%. Acute myocarditis was confirmed with myocardial biopsy. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, vasopressor inotropes, intra-aortic balloon pump and mechanical ventilation, and was discharged 21 days later. One year after diagnosis, the patient was asymptomatic. The eosinophilic cell count was normal. Follow-up MRI at one year showed LVEF of 40% with persistent delayed enhancement. Cardiac involvement by CSS requires immediate therapy with corticosteroids and cyclophosphamide, which may allow recovery of the cardiac function.