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慢性炎症性肺疾病中的肺动脉高血压性病变(作者译)

[Hypertensive lesions of pulmonary arteries in chronic inflammatory lung diseases (author's transl)].

作者信息

Leu H J, Rüttner J R, Hürlimann P

出版信息

Virchows Arch A Pathol Anat Histol. 1979 Aug 23;383(3):283-92. doi: 10.1007/BF00430247.

Abstract

150 cases of chronic inflammatory lung diseases of unknown aetiology and assumed hyperergic (immuno-reactive) pathogenesis were examined for hypertensive pulmonary arterial lesions and for chronic cor pulmonale. Hypertensive lesions of the small pulmonary arteries were found in more than half of the cases with chronic disorders of long duration, but were inconspicuous in diseases of acute progressive character. Hypertensive lesions were found regularly in chronic interstitial pneumonia, frequently in scleroderma and rheumatoid arthritis and occasionally in dermatomyositis and disseminated lupus erythematosus. Chronic Cor pulmonale occurred in 16% of the cases with hypertensive arterial lesions of grade I (hypertrophy of media) and in 50% of grade II/III (hypertrophy of media and intimal fibrosis). Interstitial lung fibrosis plays an important role in the pathogenesis of cor pulmonale: two thirds of the cases with interstitial lung fibrosis had developed cor pulmonale and all the cases with cor pulmonale also had interstitial lung fibrosis. Hypertensive arterial lesions of grade IV-VI according to Heath and Edwards (angiitis, plexogenic and angiomatoid lesions) have been described in severe cases of pulmonary hypertension (congenital cardiac shunts, primary pulmonary hypertension). In secondary forms of pulmonary hypertension, as represented by our material, these changes are of little importance.

摘要

对150例病因不明且假定为高敏性(免疫反应性)发病机制的慢性炎症性肺部疾病患者进行了高血压性肺动脉病变和慢性肺源性心脏病的检查。在病程较长的慢性疾病患者中,超过半数发现有小肺动脉的高血压性病变,但在急性进行性疾病中并不明显。在慢性间质性肺炎中经常发现高血压性病变,在硬皮病和类风湿关节炎中较为常见,在皮肌炎和播散性红斑狼疮中偶尔发现。在I级高血压性动脉病变(中膜肥厚)的病例中,16%发生了慢性肺源性心脏病,在II/III级(中膜肥厚和内膜纤维化)的病例中,50%发生了慢性肺源性心脏病。间质性肺纤维化在肺源性心脏病的发病机制中起重要作用:三分之二的间质性肺纤维化病例发展为肺源性心脏病,所有肺源性心脏病病例也都有间质性肺纤维化。根据希思和爱德华兹的分级标准,IV-VI级高血压性动脉病变(血管炎、丛状和血管瘤样病变)已在重度肺动脉高压病例(先天性心脏分流、原发性肺动脉高压)中被描述。在以我们的病例为代表的继发性肺动脉高压形式中,这些变化并不重要。

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