嗜伊红性肉芽肿多发性血管炎

查格-施特劳斯综合征(Churg–Strauss syndrome (CSS)、也称为"嗜酸性韦格纳肉芽肿""(eosinophilic granulomatosis with polyangiitis)或"过敏性肉芽肿"(allergic granulomatosis))是一种自体免疫性疾病状况，如有气道过敏(过敏体质(Atopy))过敏史的人会导致小型和中型的血管(血管炎)炎症。它通常体现出三个阶段。早期(前驱症状(Prodrome))阶段的特点是气道炎症；几乎所有的患者都会经历哮喘和/或过敏性鼻炎。第二阶段的特征是异常高的"嗜酸性粒细胞"(eosinophils)(嗜伊红细胞增多(Hypereosinophilia))，这会导致组织损伤，最常伤到肺部和消化道。第三阶段包括血管炎，其最终可以导致细胞死亡，并且可以造成生命的威胁性。
查格-施特劳斯综合征的有效治疗需要抑制免疫系统的药物。通常是糖皮质激素，其次是其他的药剂如环磷酰胺、或硫唑嘌呤。
"查格-施特劳斯综合征"它赋予著于1951年最先发现这个病症的两位医生"查格-施特劳斯"的名字。
症状和病征.
过敏性阶段.
前驱阶段的特点是过敏。 On average, asthma develops from three to nine years before the other signs and symptoms.
The allergic rhinitis may produce symptoms such as rhinorrhea and nasal obstruction, and the formation of nasal polyps that require surgical removal, often more than once. Sinusitis may also be present.-->
嗜酸性粒细胞阶段.
第二阶段特征是在血液和组织中的含有异常高水平的嗜酸性粒细胞(Hypereosinophilia、一种白血球)。 The signs and symptoms of hypereosinophilia may include weight loss, night sweats, asthma, cough, abdominal pain, and gastrointestinal bleeding. Fever and malaise are often present.
The eosinophilic stage can last months or years, and its symptoms can disappear, only to return later. Patients may experience the third stage simultaneously.-->
血管炎阶段.
Severe complications may arise. Blood clots may develop within the damaged arteries in severe cases, particularly in arteries of the abdominal region, which is followed by infarction and cell death, or slow atrophy. Many patients experience severe abdominal complaints; these are most often due to peritonitis and/or ulcerations and perforations of the gastrointestinal tract, but occasionally due to acalculous cholecystitis or granulomatous appendicitis.
The most serious complication of the vasculitic stage is heart disease, which is the cause of nearly one-half of all deaths in patients with Churg–Strauss syndrome. Among heart disease-related deaths, the most usual cause is inflammation of the heart muscle caused by the high level of eosinophils, although some are deaths to inflammation of the arteries that supply blood to the heart or pericardial tamponade. Kidney complications have been reported as being less common.-->
诊断.
诊断标记包括嗜酸性粒细胞、及受感染组织的肉芽肿(Granuloma)，以及抗中性粒细胞之抗中性白细胞胞质抗体(Anti-neutrophil cytoplasmic antibody)。1990年美国风湿病学院(American College of Rheumatology)为"查格-施特劳斯氏综合征"的诊断列出了这些标准：
以分类效果而言，患者在六项标准中如超过四项则可以判定患有查格-施特劳斯氏综合征(CSS)。六项标准的任何四项或更多项显现则产生85％的灵敏度及99.7％的特异性。
风险分层.
法国血管炎研究小组已经开发出五点系统("五因素分数")预测查格-施特劳斯氏综合征的死亡风险，且应用临床上。这些因素为：
历史背景.
查格-施特劳斯氏综合征最早在1951年纽约市西奈山医院(Mount Sinai Hospital (Manhattan))由病理学家雅各·查格(1910-2005)及罗德·施特劳斯(1913-1985)所提出。 As a result, they proposed that these cases were evident of a different disease entity, which they referred to as "allergic granulomatosis and angiitis".-->
著名案例.
Umaru Musa Yar'Adua, the president of Nigeria from 2007–2010, reportedly suffered from Churg–Strauss syndrome and died in office of complications of the disease.
DJ and author Charlie Gillett was diagnosed with Churg-Strauss in 2006; he died four years later.
Japanese ski jumper Taku Takeuchi, who won the bronze medal in the team competition, suffers from the disease and competed at the Sochi Olympics less than a month after being released from hospital treatment.-->