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特发性慢性嗜酸性粒细胞性肺炎

Idiopathic chronic eosinophilic pneumonia.

作者信息

Marchand Eric, Cordier Jean-François

机构信息

Université Catholique de Louvain, Cliniques Universitaires de Mont-Godinne, Service de Pneumologie, Yvoir, Belgium.

出版信息

Semin Respir Crit Care Med. 2006 Apr;27(2):134-41. doi: 10.1055/s-2006-939516.

Abstract

Idiopathic chronic eosinophilic pneumonia is a rare disorder of unknown cause with nonspecific respiratory and systemic symptoms but rather characteristic peripheral alveolar infiltrates on imaging. The disorder is highly responsive to oral corticosteroid therapy. However, relapses are frequent when tapering or after stopping treatment. Moreover, some patients develop severe asthma at some time in the follow-up. The high incidence of relapses and prevalence of severe asthma is responsible for the great proportion of patients with idiopathic chronic eosinophilic pneumonia who require prolonged oral corticosteroid therapy. There are tight links between asthma and idiopathic chronic eosinophilic pneumonia. These links might help in the comprehension of the pathogenesis of both diseases. Interestingly, there might exist a continuum between hypereosinophilic asthma, idiopathic chronic eosinophilic pneumonia, and the Churg-Strauss syndrome.

摘要

特发性慢性嗜酸性粒细胞性肺炎是一种病因不明的罕见疾病,具有非特异性的呼吸道和全身症状,但影像学上有相当典型的外周肺泡浸润。该疾病对口服糖皮质激素治疗反应良好。然而,在逐渐减量或停止治疗后复发频繁。此外,一些患者在随访过程中的某个时候会发展为重度哮喘。复发的高发生率和重度哮喘的患病率导致很大一部分特发性慢性嗜酸性粒细胞性肺炎患者需要长期口服糖皮质激素治疗。哮喘与特发性慢性嗜酸性粒细胞性肺炎之间存在紧密联系。这些联系可能有助于理解这两种疾病的发病机制。有趣的是,高嗜酸性粒细胞性哮喘、特发性慢性嗜酸性粒细胞性肺炎和变应性肉芽肿性血管炎之间可能存在连续性。

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