Müller K M, Fenyves A
Institut für Pathologie an den Berufsgenossenschaftlichen Krankenanstalten Bergmannsheil, Universitätsklinik, Bochum.
Pneumologie. 1991 Jan;45(1):23-7.
Ceelen's disease has been considered after the first description as a rather well defined nosological entity with severe pathological findings in the kind of a "brownish induration of the lung without a detectable extrapulmonary circulation". Nowadays this disease enter into the general discussion of uncertain pulmonary bleeding mechanism. So far not further characterized structural changes in the pleura in an open lung biopsy of a 28-year-old woman with Ceelen's disease are reported. Basically the alterations consists of disruption of the elastic laminae associated with a giant cell reaction with incorporation of elastica fragments. In a survey of mainly pathological anatomical reports on Ceelen's disease the hypothesis of a primary alteration of the elastic tissue is confronted with the hypothesis of a primary not yet in detail characterised defect of the alveolar-capillary entity and brought in relation to the particular findings in the pleura.
在首次描述后，塞伦病被认为是一种定义较为明确的病种，具有严重的病理表现，即“肺部呈褐色硬结，未检测到肺外循环”。如今，这种疾病已进入关于不明肺出血机制的一般性讨论中。迄今为止，尚未有关于一名患有塞伦病的28岁女性在开放性肺活检中胸膜结构变化的进一步特征报道。基本上，这些改变包括弹性纤维层的破坏，并伴有巨细胞反应以及弹性组织碎片的融合。在一项主要关于塞伦病的病理解剖报告调查中，弹性组织原发性改变的假说与肺泡 - 毛细血管单位原发性但尚未详细描述的缺陷假说相对立，并与胸膜的特定发现相关联。
