Churg Strauss Syndrome

Harshna Richard Qadir
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Abstract

Churg Strauss syndrome (CSS) also known as Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition characterized by asthma, high levels of eosinophils (a type of white blood cell that helps fight infection), and inflammation of small to medium sized blood vessels (vasculitis).1 The inflamed vessels can affect various organ systems including the lungs, gastrointestinal tract, skin, heart and nervous system. No known cause of disease is found but believe to be an auto immune disorder and genetic factors and allergies play vital role to trigger the disease. CSS has 3 stages, with its own clinical manifestations. It’s not necessary that all patients with CSS will go through all 3 stages and they also do not manifest in same order always. The first stage is an allergic stage presents with respiratory problems, second stage is eosinophilic stage characterized by intra organ infiltration of eosinophils and final stage is a vasculitic stage with swollen lypmph nodes, joint pain, necrotizing inflammation of small vessels. Morbidity rate is high and it also need long term monitoring.
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丘格·施特劳斯综合症
Churg Strauss综合征(CSS)也被称为嗜酸性肉芽肿病伴多血管炎(EGPA),是一种罕见的疾病,以哮喘、高水平嗜酸性粒细胞(一种帮助抵抗感染的白细胞)和中小型血管炎症(血管炎)为特征发炎的血管会影响各种器官系统,包括肺、胃肠道、皮肤、心脏和神经系统。目前还没有发现病因,但相信是一种自身免疫紊乱,遗传因素和过敏在引发疾病方面起着至关重要的作用。CSS分为3期,各有其临床表现。并不是所有的CSS患者都必须经历所有的三个阶段,而且它们也不总是以相同的顺序出现。第一阶段为过敏期,表现为呼吸问题;第二阶段为嗜酸性粒细胞期,表现为器官内嗜酸性粒细胞浸润;最后阶段为血管增生期,表现为淋巴结肿胀、关节疼痛、小血管坏死性炎症。发病率高,需要长期监测。
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