Marchand E, Cordier J F
Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERMOP), Hôpital Louis Pradel, Lyon (Bron), France.
Rev Mal Respir. 2006 Sep;23(4 Suppl):13S99-108.
Idiopathic chronic eosinophilic pneumonia (ICEP) is one of the hypereosinophilic lung diseases.
ICEP is a rare disease of unknown cause that combines non-specific respiratory and general symptoms with predominantly peripheral radiological infiltrates. The presence of blood and alveolar eosinophilia points strongly to the diagnosis. ICEP is very sensitive to systemic corticosteroids but relapses are common following stopping treatment or reducing the dose. A background of asthma is commonly found and many patients with ICEP develop severe asthma which, together with the relapses, often necessitates prolonged systemic corticosteroid treatment. The long term prognosis, however, remains excellent.
The role of inhaled corticosteroids in non-asthmatic patients remains uncertain and should be evaluated further. The links between asthma and ICEP could lead to a better understanding of the mechanisms underlying hypereosinophilic lung diseases.
ICEP is a rare disease that is important to recognise on account of its potentially disabling nature and its good response to corticosteroid treatment though long term maintenance is some times necessary on account of relapses or the development of severe asthma.
特发性慢性嗜酸性粒细胞性肺炎(ICEP)是嗜酸性粒细胞增多性肺部疾病之一。
ICEP是一种病因不明的罕见疾病,其非特异性呼吸道症状和全身症状与主要的外周放射学浸润相结合。血液和肺泡嗜酸性粒细胞增多强烈提示诊断。ICEP对全身用皮质类固醇非常敏感,但停药或减药后复发很常见。通常会发现有哮喘病史,许多ICEP患者会发展为重度哮喘,这与复发一起,常常需要长期全身用皮质类固醇治疗。然而,长期预后仍然良好。
吸入性皮质类固醇在非哮喘患者中的作用仍不确定,应进一步评估。哮喘与ICEP之间的联系可能有助于更好地理解嗜酸性粒细胞增多性肺部疾病的潜在机制。
ICEP是一种罕见疾病,鉴于其潜在的致残性以及对皮质类固醇治疗的良好反应,尽管有时由于复发或重度哮喘的发展需要长期维持治疗,但认识到这一点很重要。