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The development of fibrous tissue in the pleura is the main cause of pleural fibrosis. It is the main indicator of remote exposure (more than 20 years before) or a short-lived and heavy exposure to asbestos. It can be either focal or diffuse process. The fibrosed pleura may fold in on itself, encasing a portion of the parenchyma or surround the lung, leading to a trapped lung. To distinguish any form of pleural fibrosis from malignancy, biopsy is required to ensure benignity. The presence of pleural fibrosis usually means that there is an increased risk of pulmonary fibrosis development.
The thickness may vary from a slight milky discoloration to a thick, white peel that encases one or both lungs. It also may be localized or widespread. Asbestos bodies and fibers can also be discovered in the fibrous tissue. Even in 15+ years after asbestos exposure (usually amphiboles) there is a big chance of development of Pleural fibrosis. Cumulative exposure to asbestos is much less important than time since first exposure.
Pleural fibrosis has the same symptoms as fibrosis in other tissues. So it can be described as the destruction of normal pleural tissue architecture and compromised function due to an excessive deposition of matrix components. Many diseases can result in pleural fibrosis. For example organised haemorrhagic effusion, tuberculous effusion, empyema or asbestos-related pleurisy. Pleural fibrosis has several ways to manifest itself. It can be either discrete localised lesions (pleural plaques) or fibrosis and diffuse pleural thickening.
Pleural fibrosis is a result of different processes, for example of interactions between profibrotic mediators and coagulation, resident and inflammatory cells. It is generally considered that subpleural fibroblast is the primary target cell for pleural fibrosis.
Sometimes pleural fibrosis can be the result of successful treatment of inflammatory reactions. Most of the time only a slight amount of scar tissue remains after complete healing of long-standing or severe inflammation. Occasionally the lung can become encased in a thick fibrous layer. It can retract the mediastinum toward the side of disease, limit chest wall motion, and impairs pulmonary function.
Pleural fibrosis can often be accompanied by calcification of the pleura. It can manifest after intrapleural hemorrhage or infection as focal, usually fenestrated, irregular plaques on the costal surfaces. Focal calcification accompanied plaque like pleural fibrosis can develope 20 or more years after asbestos exposure. It usually affects the diaphragmatic pleura.
There is another factor that is called the growth factors, that is currently applicable in a clinical setting. It is very important to understand the biology of these growth factors because it can allow to inhibit pleural (and peritoneal) adhesion/fibrosis or to create pleurodesis.
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