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 …     

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

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

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.     

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     

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.     

Montelukast-associated Churg-Strauss syndrome

A 68-year-old asthmatic presented markedly unwell with arthralgia, mononeuritis multiplex, peripheral neuropathy, and eosinophilia. His past medical history included perennial rhinitis, and nasal polyps. Three months prior to admission his prednisolone was stopped and Montelukast was started. The diagnosis of Montelukast-associated Churg-Strauss syndrome was made. The drug was stopped and steroids started with general improvement and reduction of eosinophilia; however, the neurological deficit persisted.     

Montelukast and Churg-Strauss syndrome

The association of leukotriene receptor antagonists and Churg-Strauss Syndrome (CSS) has been recognised for several years. However, whether these drugs have a direct pathogenic role remains controversial. The present case describes an asthmatic patient, who developed severe obstructive symptoms and progressive heart failure after two sequential exposures to montelukast. As the patient exhibited a markedly raised blood eosinophil count with diffuse infiltrates on chest x-ray and signs of myocarditis, CSS was suspected. The disease was confirmed by open lung biopsy. The symptoms improved rapidly after administration of high dose immunosuppression with methylprednisolone and cyclophosphamide. This case is noteworthy because the time course of events strongly suggests a direct aetiological role for montelukast in the development of CSS. The pathophysiological mechanism of the association remains unknown.     

Myopericarditis in Churg-Strauss syndrome

Churg-Strauss syndrome is a disseminated vasculitis with multisystem involvement, characterized by necrotizing arteritis, eosinophilic infiltration, and extravascular granuloma formation. In as many as 60% of all cases, the heart may be affected. We describe a 30-year-old man in whom pericarditis was followed by the development of a large pericardial effusion, with evidence of impaired right and left ventricular function. The patient had a 5-year history of asthma. Early therapy with high-dose prednisolone and azathioprine led to resolution of the pericardial effusion and prevented a further reduction in biventricular function.     

Childhood Churg-Strauss syndrome.

Churg-Strauss syndrome or allergic granulomatosis and angiitis is a vasculitis that is found in adults, but is extremely rare in children. We describe a 14-year-old boy who presented with prolonged fever, weight loss, sinusitis, myalgia and arthralgia, testicular pain, pulmonary infiltrations, pericardial effusion, peripheral neuropathy, and eosinophilia. Muscle biopsy showed necrotizing arteritis with eosinophil infiltration. His clinical course was complicated by several seizures secondary to cerebral vasculitis and severe asthma, resulting in death. The clinical features and outcomes of childhood Churg-Strauss syndrome are reviewed.     

Churg-Strauss syndrome related to montelukast

A young male with complaints of cough, dyspnea and hemoptysis was admitted. He was using fluticasone propionate and salmeterol for two years for his asthma. Leukotriene receptor antagonist was prescribed two weeks prior to his admission and no reduction of his inhaled steroid therapy was performed. Eosinophil count was detected as 1460/mm3 (15%) and immunoglobulin E level was 547 IU/mL. Thorax computerized tomography revealed patchy infiltration. Increased eosinophilic inflammation were detected in bronchoalveolar lavage fluid and transbronchial biopsy. He received prednisolone treatment for Churg-Strauss syndrome. Improvement was observed on three months follow up period. He has no complaint in his follow up.     

Heart transplantation for Churg-Strauss syndrome

A patient with heart failure caused by Churg-Strauss syndrome was successfully treated with transplantation. The case was unusual because there was little evidence of Churg-Strauss syndrome in the lung. The patient remains well on standard transplant immunotherapy.