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吸入性肺纤维化:矽肺

[Pulmonary fibrosis caused by inhalation: silicosis].

作者信息

Ortuño Pacheco G

机构信息

Cátedra de Anatomía Patológica, Universidad de Murcia.

出版信息

Rev Med Univ Navarra. 1998 Oct-Dec;42(4):208-19.

Abstract

Silicosis is an important disease not only for its prevalence and the degree of pulmonary insufficiency it entails but also because it provides a natural model of interstitial fibrotic disease in the lung which is of known origin. This can, in turn, help us understand the pathogenic nature of a great number of pulmonary fibroses whose cause is unknown. The fifty postmortem studies which we describe correspond to miners who had worked in underground mines in the mountainous region near Cartagena (SE Spain) for times ranging from 5 to 36 years. The autopsies showed that they had been exposed to dust containing abundant metallic particles, particularly iron oxide (mixed dust). Although the pathogenic action was related with silica, it was also clearly modified by the composition of the dust associated with it. The basic lesions which are produced in the lung after inhalation of silica (< 5 microns diameter) are coniosis, fibroconiosis and sclerohyalinosis. The sclerohyalino nodules are characterized by abundant collagenization and hyalinization, different types of which can be distinguished according to the disposition of the collagenous fibres. Nodular lesions gradually grow in size even when exposure to dust has ceased. As they grow they get nearer until they join to form conglomerate masses. When the diameter exceeds 3 cm this is called massive fibrosis. Pulmonary tuberculosis was found in 27% of cases. This took the form of lesions, which could be associated to or independent of silicotic lesions. Besides evolutive nodular lesions, a patient suffering from silicosis may show other unspecific lesions which must be correctly evaluated for a more correct clinical-pathological assessment, since, clinically, the respiratory function may be profoundly affected although such silicotic damage may be not very noticeable by radiological examination. Silicosis of the liver and spleen was not infrequent in the autopsies carried out, with basic lesions in all evolutive states being observed, the most evolved in the spleen. This means that silicosis should be considered as a systemic illness.

摘要

矽肺是一种重要的疾病,不仅因其患病率和所导致的肺功能不全程度,还因为它为已知病因的肺间质纤维化疾病提供了一个自然模型。这反过来又有助于我们理解大量病因不明的肺纤维化的致病本质。我们描述的五十例尸检研究对象是在卡塔赫纳(西班牙东南部)附近山区的地下矿井工作了5至36年的矿工。尸检显示,他们接触过含有大量金属颗粒,特别是氧化铁的粉尘(混合粉尘)。虽然致病作用与二氧化硅有关,但也明显受到与之相关的粉尘成分的影响。吸入二氧化硅(直径<5微米)后在肺部产生的基本病变是尘肺、纤维尘肺和硬化性透明变性。硬化性透明变性结节的特征是大量胶原化和透明变性,根据胶原纤维的排列可区分不同类型。即使停止接触粉尘,结节性病变的大小也会逐渐增大。随着它们的生长,彼此靠近直至融合形成团块。当直径超过3厘米时,这被称为大块纤维化。27%的病例中发现了肺结核。其表现形式为病变,可与矽肺病变相关或独立存在。除了进行性结节性病变外,矽肺患者可能还会出现其他非特异性病变,为了进行更准确的临床病理评估,必须对这些病变进行正确评估,因为在临床上,呼吸功能可能会受到严重影响,尽管通过放射学检查这种矽肺损害可能不太明显。在进行的尸检中,肝脾矽肺并不少见,观察到了各个发展阶段的基本病变,脾脏中的病变最为严重。这意味着矽肺应被视为一种全身性疾病。

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