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[哈曼-里奇综合征]

[Hamman-Rich syndrome].

作者信息

Braun A V, Pilipenko V Z, Petskalev A Z, Blazhko A Z

出版信息

Arkh Patol. 1979;41(2):59-62.

PMID:426650
Abstract

An observation of Hamman-Rich syndrome in a man of 47 with an acute, rapidly progressive course due to an immunoconflict situation in the lungs is presented. This situation is characterized by marked changes of the microcirculatory bed of interalveolar septae, with increased permeability, productive-desquamative alveolitis and formation of hyalin membranes, lymphoid-macrophage cell reaction of diffuse fibrosis. The peculiar features of this case include not only the involvement of the lungs but also the occurrence of generalized productive vasculitis with the involvement of the myocardium and the kidneys which, alongside with considerable immune alterations in the spleen and lymph nodes, confirm the hypothesis of the systemic autoaggression nature of this disease.

摘要

报告了一名47岁男性的Hamman-Rich综合征观察病例,该病例因肺部免疫冲突情况呈急性、快速进展病程。这种情况的特征是肺泡间隔微循环床有明显变化,包括通透性增加、增殖-脱屑性肺泡炎和透明膜形成、弥漫性纤维化的淋巴-巨噬细胞反应。该病例的独特之处不仅在于肺部受累,还在于出现了累及心肌和肾脏的全身性增殖性血管炎,这与脾脏和淋巴结中相当程度的免疫改变一起,证实了该疾病具有系统性自身攻击性质的假说。

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