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[播散性红斑狼疮中的致死性急性肺炎]

[Fatal acute pneumonopathies in disseminated lupus erythematosus].

作者信息

Kerbourc'h J F, Cledes J, Garre M, Zabbe C, Gouva S, Clavier J

机构信息

Service de Pneumologie et Oncologie broncho-pulmonaire, CHRU Augustin Morvan, Brest.

出版信息

Rev Pneumol Clin. 1988;44(1):6-13.

PMID:3387779
Abstract

Two cases of systemic lupus erythematosus (SLE) complicated by pneumonia which resulted in death are reported. The first patient, a 21-year old woman, died of acute diffuse lupus pneumonia; the initial and unusual radiological image of "multiple balloons" progressed within 2 months to terminal interstitial fibrosis. The second patient, a 60-year old woman, died of infection on an interstitial pneumonia which turned into severe fibrosis within 16 months. Acute or chronic lupus pneumonia is uncommon, but it may follow a very serious course. Clinically, true lupus pneumonia must be distinguished from all other types of lung involvement in SLE, such as infection, pulmonary haemorrhage or oedema, iatrogenic pathology, thromboembolic disease, etc. The pathogenetic mechanism of pulmonary lesions directly related to SLE is obscure, although some lung biopsy specimens have shown positive immunofluorescence. Concerning treatment, the initial response to corticosteroid therapy is usually very good, especially in the acute forms of the disease. However, in severe cases immunosuppressive drugs or even plasma exchanges must be added to steroids. For treatment to be rapidly initiated the diagnostic procedures must be completed in the early stages of the disease, involving, when necessary, surgical lung biopsy.

摘要

报告了两例系统性红斑狼疮(SLE)合并肺炎导致死亡的病例。首例患者为一名21岁女性,死于急性弥漫性狼疮肺炎;最初不寻常的“多个气囊”放射影像在2个月内进展为终末期间质纤维化。第二例患者为一名60岁女性,死于间质性肺炎感染,该病在16个月内发展为严重纤维化。急性或慢性狼疮肺炎并不常见,但病情可能非常严重。临床上,必须将真正的狼疮肺炎与SLE中所有其他类型的肺部受累情况区分开来,如感染、肺出血或水肿、医源性病变、血栓栓塞性疾病等。尽管一些肺活检标本显示免疫荧光呈阳性,但与SLE直接相关的肺部病变的发病机制尚不清楚。关于治疗,对皮质类固醇治疗的初始反应通常非常好,尤其是在疾病的急性形式中。然而,在严重病例中,必须在类固醇治疗的基础上加用免疫抑制药物甚至进行血浆置换。为了迅速开始治疗,必须在疾病的早期阶段完成诊断程序,必要时进行外科肺活检。

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