Department of Pediatrics, Asan Medical Center Children's Hospital, Seoul, Korea.
Allergy Asthma Immunol Res. 2012 Jul;4(4):240-4. doi: 10.4168/aair.2012.4.4.240. Epub 2012 Mar 9.
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.
一些表现为轻度呼吸道感染症状的儿童被诊断为未分类间质性肺炎伴纤维化。他们的临床和影像学表现与急性间质性肺炎相似，但在病理发现上存在一些差异。未分类间质性肺炎伴纤维化的特征是组织学发现肺泡损伤和细支气管破坏呈小叶中心分布，伴有细支气管闭塞。本报告描述了两例不同系列的家族性未分类间质性肺炎伴纤维化病例，它们几乎同时在春季发病。一些个别病例表现为不明原因的快速进展性呼吸衰竭，具有相似的临床过程和类似的影像学和病理学特征，包括肺纤维化。每个家庭成员几乎同时在春季受到影响，两名患者检测到不同类型的病毒，所有成员均未感染细菌、环境和职业因素、药物和辐射。这些发现提示病毒感染和/或与病毒感染相关的过程，如过度或改变的免疫反应，或未知的吸入性环境因素在未分类间质性肺炎伴纤维化的发病机制中起作用。
