Two series of familial cases with unclassified interstitial pneumonia with fibrosis.
Two series of familial cases with unclassified interstitial pneumonia with fibrosis.
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DOI：
10.4168/aair.2012.4.4.240
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发表时间：
2012-07
期刊：
影响因子：
--
通讯作者：
Hong SJ
中科院分区：
文献类型：
--
作者：
Lee E;Seo JH;Kim HY;Yu J;Song JW;Park YS;Jang SJ;Do KH;Kwon J;Park SW;Park JH;Hong SJ
几名出现轻度呼吸道感染症状的儿童被诊断为未分类的间质性肺炎伴纤维化。其临床和影像学表现与急性间质性肺炎相似，但病理表现有一定差异。未分类的间质性肺炎伴纤维化的特征是肺泡损伤和细支气管破坏伴细支气管闭塞的小叶中心分布的组织学表现。本报告描述了两个不同系列的家族性病例未分类间质性肺炎与纤维化，其中开发几乎同时在春季。一些个体病例显示原因不明的快速进行性呼吸衰竭，具有可比的临床病程和相似的放射学和病理学特征，包括肺纤维化。每个家庭成员在春季几乎同时受到影响，在两名患者中检测到不同类型的病毒，所有成员对细菌感染、环境和职业因子、药物和辐射均呈阴性。这些发现表明病毒感染和/或与病毒感染相关的过程，如免疫反应增强或改变，或未知的吸入性环境因子在未分类的间质性肺炎伴纤维化的发病机制中。
Several children presenting with mild symptoms of respiratory tract infection were diagnosed with unclassified interstitial pneumonia with fibrosis. Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized by histological findings of centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration. This report describes two different series of familial cases of unclassified interstitial pneumonia with fibrosis, which developed almost simultaneously in the spring. Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis. Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation. These findings implicate a viral infection and/or processes related to a viral infection, such as an exaggerated or altered immune response, or an unknown inhaled environmental agent in the pathogenesis of unclassified interstitial pneumonia with fibrosis.