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[慢性嗜酸性粒细胞性肺炎或卡林顿病]

[Chronic eosinophilic pneumonia or Carrington's disease].

作者信息

Durieu J, Wallaert B, Tonnel A B

机构信息

Service de Pneumologie et Immuno-allergologie, Hôpital Albert-Calmette, CHRU de Lille.

出版信息

Rev Mal Respir. 1993;10(6):499-507.

PMID:8122016
Abstract

Chronic eosinophilic pneumonia (CEP) was described by Carrington in 1969. It is twice as common in women as it is in men and is probably most common at middle age. Symptoms usually associated fever, weight loss, fatigue and dyspnoea. Extra-respiratory manifestations are rare but may involve many organs. Classical radiological findings are diffuse alveolar infiltrates, with a peripheral predominance which explains the classic description of a "negative of pulmonary edema". Peripheral blood eosinophilia is present in 90%. The inflammatory syndrome with an eosinophilic alveolitis is constant. Pulmonary function tests show a restrictive pattern with depressed DLCO and hypoxemia. In atypical cases, proof of diagnosis is supported by lung biopsies that show intra alveolar and interstitial accumulation of eosinophils in peripheral air spaces. Areas of bronchiolitis obliterans are also frequently seen. In some atypical forms of chronic eosinophilic pneumonia, other disorders have to be discussed, like bronchiolitis obliterans with an organizing pneumonia or cryptogenetic organizing pneumonia and even tuberculosis. After corticosteroid therapy, the resolution occurs very rapidly but relapses are frequent. Steroid-dependence is seen in 20 to 30% of patients.

摘要

慢性嗜酸性粒细胞性肺炎(CEP)由卡林顿于1969年首次描述。女性发病率是男性的两倍,发病高峰年龄可能在中年。症状通常包括发热、体重减轻、疲劳和呼吸困难。肺外表现罕见,但可能累及多个器官。典型的影像学表现为弥漫性肺泡浸润,以外周为主,这就是“肺水肿阴性”这一经典描述的由来。90%的患者外周血嗜酸性粒细胞增多。伴有嗜酸性粒细胞性肺泡炎的炎症综合征持续存在。肺功能检查显示限制性通气模式,伴有弥散功能(DLCO)降低和低氧血症。在非典型病例中,肺活检显示外周气腔内肺泡内和间质嗜酸性粒细胞聚集可支持诊断。闭塞性细支气管炎区域也很常见。在一些非典型的慢性嗜酸性粒细胞性肺炎中,必须考虑其他疾病,如伴有机化性肺炎的闭塞性细支气管炎或隐源性机化性肺炎,甚至是肺结核。皮质类固醇治疗后,病情缓解非常迅速,但复发频繁。20%至30%的患者会出现类固醇依赖。

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