Churg-Strauss syndrome

Churg-Strauss syndrome is a vasculitis associated with asthma and eosinophilia. Respiratory involvement is marked by generally severe and often steroid-dependent late-onset asthma associated with allergic rhinitis and sometimes nasal polyposis and recurrent sinusitis. Asthma generally precedes the systemic vasculitis by a few years. General signs, eosinophilic gastroenteritis, peripheral multiplex neuropathy, cutaneous vasculitis, nephropathy, or arthromyalgia, predominate. Cardiac involvement is often silent but of severe prognosis. The chest X-ray usually shows irregularly delimited and sometimes labile infiltrates. Perinuclear antineutrophil cytoplasmic autoantibodies (ANCA) are found in two-thirds of the patients and strongly suggest the diagnosis. Clinically, the diagnosis is established by the presence of asthma, peripheral eosinophilia > 1.5 G/L, and systemic vasculitis involving at least two extra-pulmonary organs. Histological confirmation is usually necessary (nerve and muscle biopsy), showing small-vessel eosinophilic vasculitis, tissue infiltration with eosinophils, and eosinophilic granulomas. Treatment includes corticosteroids, which should be associated with immunosuppressive agents (cyclophosphamide) in severe cases.     

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 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.     

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 in patients receiving montelukast as treatment for asthma

STUDY OBJECTIVES: We previously reported eight patients who developed Churg-Strauss syndrome in association with zafirlukast treatment for asthma and postulated that the syndrome resulted from unmasking of a previously existing condition due to corticosteroid withdrawal and not from a direct drug effect. The availability of montelukast, a new leukotriene receptor antagonist with a different molecular structure, permitted us to test this hypothesis. Our goals were to ascertain whether the Churg-Strauss syndrome developed in patients taking montelukast and other novel asthma medications, and to describe potential mechanisms for the syndrome. DESIGN: Case series. SETTING: Outpatient and hospital practices of pulmonologists in the United States and Belgium. PATIENTS: Four adults (one man, three women) who received montelukast as treatment for asthma; two women who received salmeterol/fluticasone therapy, but not montelukast. RESULTS: Churg-Strauss syndrome developed in the four asthmatic patients who received montelukast. In each case, there was a long history of difficult-to-control asthma characterized by multiple exacerbations that had required frequent courses of oral systemic corticosteroids or high doses of inhaled corticosteroids for control. Two other asthmatics who received fluticasone and salmeterol but not montelukast therapy developed the same syndrome with tapering doses of oral or high doses of inhaled corticosteroids. CONCLUSIONS: The occurrence of Churg-Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients.     

白三烯拮抗剂与变应性肉芽肿性血管炎的关系

随着白三烯拮抗剂应用于哮喘治疗以来,国内外越来越多的文献报道了使用白三烯拮抗剂后出现变应性肉芽肿性血管炎的病例.本文就变应性肉芽肿性血管炎、白三烯拮抗剂及两者间关系的研究进展做了相应综述介绍.     

Fulminant eosinophilic endomyocarditis in an asthmatic patient treated with pranlukast after corticosteroid withdrawal

Several cases of eosinophilic conditions including Churg-Strauss syndrome have been reported in association with the use of cysteinyl leukotriene receptor antagonists, including zafirlukast, montelukast, and pranlukast, in asthmatic patients. The case of a 26 year old woman with a three year history of asthma, rhinitis, and nasal polyps is reported in whom eosinophilia, pulmonary infiltrates, and fulminant eosinophilic endomyocarditis accompanied by cardiogenic shock developed during pranlukast treatment after corticosteroid withdrawal. Acute necrotising eosinophilic endomyocarditis was confirmed by endomyocardial biopsy. The patient recovered after intensive treatment, including mechanical assistance involving intra-aortic balloon pumping and steroid pulse therapy, along with the discontinuation of pranlukast. It is recommended that careful attention must be paid to signs of a systemic eosinophilic condition or even fulminant eosinophilic myocarditis in asthmatic patients who have begun treatment with antileukotriene drugs following withdrawal of steroids.


Keywords: fulminant eosinophilic endomyocarditis; asthma; pranlukast     

Churg Strauss syndrome after introducing oral steroid to inhaler: a report of three cases

The tetrad of bronchial asthma, severe sinusitis, nasal polyp, eosinophilia, and systemic vasculitis is the main feature of allergic granulomatosis and angitis (Churg- Strauss Syndrome). This vasculitis is usually seen idiopathic in patients with a long history of asthma; oral steroids using steroid inhalers, vaccination and desensitization might be triggering factors. Drugs such as leukotriene receptor antagonists (LTRAS), penicillin, sulphonamides, anticonvulsants and thiazides have also been implicated. By presenting the cases in this article, the authors suggest that some cases of CSS may be partially or totally suppressed by corticosteroid therapy of asthma for long periods and replacing oral steroid by inhaler will reveal a pathologic condition of CSS, called frustes CSS forms. We report three subjects with asthma who had been receiving previously multiple corticosteroid courses for control, but when systemic corticosteroids were discontinued or switched over to steroid inhaler, the patients developed a similar syndrome.     

Churg-Strauss syndrome associated with omalizumab

Reports have suggested that the leukotriene receptor antagonists may cause Churg-Strauss syndrome following the tapering of oral corticosteroids or even in patients not treated with corticosteroids. We report a patient not treated with long-term corticosteroids and who developed Churg-Strauss syndrome with myocarditis and central nervous system involvement following the initiation of omalizumab. Given the possible role of omalizumab in the development of this vasculitis in our patient, we recommend careful monitoring of the appearance of Churg-Strauss symptoms and rising eosinophil count in patients treated with omalizumab.     

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.     

Churg-Strauss syndrome

Churg-Strauss syndrome was originally called "allergic granulomatosis and angiitis," describing the combination of eosinophilic inflammation, extravascular granulomas, and necrotizing vasculitis occurring in patients with severe asthma. It is now classified as a small-vessel vasculitis and, together with Wegener's granulomatosis and microscopic polyangiitis, as one of the vasculitides associated with antineutrophil cytoplasmic autoantibodies (ANCA). Glucocorticoid-sparing agents used in the treatment of asthma, such as leukotriene receptor antagonists, may unmask this particular form of vasculitis as oral glucocorticoids are withdrawn. ANCA occur in 40-75% of patients with active disease and typically react with myeloperoxidase. Patients' symptoms are defined by various degrees of eosinophilic inflammation and necrotizing vasculitis, which may affect any organ. On presentation, Churg-Strauss syndrome needs to be differentiated from other eosinophilic pneumonias, idiopathic hypereosinophilic syndrome, and Wegener's granulomatosis and microscopic polyangiitis. Churg-Strauss syndrome remains a rare disease with a poorly understood pathogenesis. Treatment consists primarily of glucocorticoids. Patients who have ANCA at the time of presentation should be treated according to the treatment principles for ANCA-associated vasculitides. However, the exact role of glucocorticoid-sparing immunosuppressive agents and treatment options for refractory disease remain poorly studied.     

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.     

Cholestatic hepatitis: an uncommon presentation of Churg-Strauss syndrome

Churg-Strauss syndrome is a rare form of severe vasculitis characterized by severe asthma and hypereosinophilia. Liver involvement is rare. We report a case of a woman with serious cholestatic hepatitis and vasculitis, without severe asthma. Marked hypereosinophilia and liver biopsy confirmed diagnosis. Churg-Strauss syndrome must be recognized because digestive involvement is very serious and affect the prognosis of this disease.     

Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature

BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.     

Churg-Strauss综合征肺损害1例并文献复习

目的 加深对Churg-Strauss综合征又名变应性肉芽肿血管炎(CSS)的认识.方法 通过对1例CSS病例的诊断、治疗及相关文献进行研究学习,分析Churg-Strauss综合征的病因、临床症状、实验室检查、影像学表现、诊断、鉴别诊断及治疗.结果 CSS病因不清,临床表现无特异性,可累及多系统器官,最常见的临床表现是哮喘及血管炎引起的肺部表现,特征性的病理表现为外周血嗜酸粒细胞增多,目前治疗首选激素,预后较好.结论 Churg-Strauss综合征是一种少见的系统性血管炎,临床缺乏特异性表现,易被误诊,当患者表现为哮喘、外周血嗜酸性粒细胞增多及肉芽肿性血管炎时,应高度怀疑此病.     

Churg-Strauss syndrome

PURPOSE OF REVIEW: Churg-Strauss syndrome is a small-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, extravascular necrotizing granulomas and hypereosinophilia. The most recent clinical studies on its pathogenesis and therapeutic management are reviewed here. RECENT FINDINGS: French and Italian clinical studies found that the clinical characteristics of patients with Churg-Strauss syndrome differed according to their antineutrophil cytoplasmic autoantibody status: cardiomyopathy predominated in antineutrophil cytoplasmic autoantibody-negative patients while necrotizing glomerulonephritis was more often observed in antineutrophil cytoplasmic autoantibody-positive patients. These histologically documented findings suggest the existence of different Churg-Strauss syndrome subtypes, characterized by the predominance of distinct pathogenetic mechanisms. To date, following the therapeutic recommendations for Churg-Strauss syndrome (i.e. corticosteroids and, when required, immunosuppressants), patient outcomes are good, with 5-year survival exceeding 90%, but often with the need to continue low-dose corticosteroids to control residual asthma. SUMMARY: The precise pathogenetic mechanisms of Churg-Strauss syndrome are only partly elucidated. Recent results suggest that antineutrophil cytoplasmic autoantibodies are probably more involved in the vasculitic manifestations of Churg-Strauss syndrome (e.g. glomerulonephritis) whereas eosinophil tissue infiltration and associated cytotoxicity would be responsible for cardiomyopathy. If confirmed, these results could support individual therapeutic stratification according to the clinical pattern. Furthermore, some patients may benefit from new biologic therapies under development, for example antiinterleukin-5 or antiimmunoglobulin E monoclonal antibodies.     

Myocardial involvement in granulomatous vasculitis (Churg-Strauss disease)

We report the case of a 44-year-old woman affected by Churg-Strauss syndrome (systemic vasculitis with eosinophilia, bronchial asthma and pulmonary infiltrations). Congestive heart failure developed, caused by severe myocardial and pulmonary involvement. Conventional treatment and steroids induced remission of symptoms.     

Churg-Strauss syndrome

Churg-Strauss syndrome is a rare diffuse vasculitis that is almost invariably accompanied by severe asthma. Although overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists. Diffuse organ involvement of Churg-Strauss syndrome, especially cardiovascular and rare involvement of the CNS and renal system, suggests a poorer prognosis than usual, and can be fatal. The cause of Churg-Strauss syndrome is unknown, but its characteristic histological findings and association with asthma distinguish it from other vasculitides. Controversy surrounds the use of asthma drugs-especially antileukotrienes--and development of the disorder. We review the epidemiological evidence for an association of drug treatment with Churg-Strauss syndrome, the diverse diagnostic and pathological criteria for this syndrome, and treatment options.     

Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre

OBJECTIVE: Churg-Strauss syndrome is a rare multisystem vasculitis of unknown aetiology. Due to the rarity of the disease, few single-centre case series have been described. The aim of this study was to evaluate a small series from a single Italian centre in order to describe the clinical features of the disease, the treatment and long-term follow-up. METHODS: Nineteen Churg-Strauss syndrome patients were selected from the medical records of all vasculitis patients attending the Immunology Unit at the Department of Internal Medicine of the University of Pisa in the decade between 1989 and 2000. Data were obtained retrospectively. RESULTS: All the patients had asthma and hypereosinophilia. As in other case series, the lungs, skin and peripheral nervous system were the most commonly involved organs. The majority of our patient received i.v. pulses of methylprednisolone followed by i.v. pulses of cyclophosphamide. The outcome and long-term follow-up were good. There were no fatalities observed in this series during the follow-up period. CONCLUSIONS: Churg-Strauss syndrome is a systemic vasculitis occurring in patients with a history of asthma and allergic rhinitis. The positive results of the treatment protocol used in this preliminary study deserve to be tested in controlled multicentre studies.