Usually, the underlying condition has to cause intense inflammation of the pleura, though it is unclear exactly how this results in fibrosis. The precise mechanisms producing the fibrosis are not entirely clear. However, research indicates a protein called Transforming Growth Factor beta (TGF-β) plays a central role in producing fibrothorax. Anti-TGF-β antibodies prevent fibrothorax in empyema in animal models. Diagnosis
A fibrothorax can typically be diagnosed by taking an appropriate medical history in combination with the use of appropriate imaging techniques such as a plain chest X-ray or CT scan. These imaging techniques can detect fibrothorax and pleural thickening that surround the lungs. The presence of a thickened peel with or without calcification are common features of fibrothorax when imaged. CT scans can more readily differentiate whether pleural thickening is due to extra fat deposition or true pleural thickening than X-rays.If a fibrothorax is severe, the thickening may restrict the lung on the affected side causing a loss of lung volume. Additionally, the mediastinum may be physically shifted toward the affected side. A reduction in the size of one side of the chest (hemithorax) on an X-ray or CT scan of the chest suggests chronic scarring. Signs of the underlying disease causing the fibrothorax are also occasionally seen on the X-ray. A CT scan may show features similar to those seen on a plain X-ray. Lung function testing typically demonstrates findings consistent with restrictive lung disease. Treatment
Non-surgical
Conservative non-surgical treatment of fibrothorax is generally done by treating its underlying cause and is reserved for milder cases.
Fibrothorax is a medical condition characterised by severe scarring (fibrosis) and fusion of the layers of the pleural space surrounding the lungs resulting in decreased movement of the lung and ribcage. The main symptom of fibrothorax is shortness of breath. There also may be recurrent fluid collections surrounding the lungs. Fibrothorax may occur as a complication of many diseases, including infection of the pleural space known as an empyema or bleeding into the pleural space known as a haemothorax.Fibrosis in the pleura may be produced intentionally using a technique called pleurodesis to prevent recurrent punctured lung (pneumothorax), and the usually limited fibrosis that this produces can rarely be extensive enough to lead to fibrothorax. The condition is most often diagnosed using an X-ray or CT scan, the latter more readily detecting mild cases. Fibrothorax is often treated conservatively with watchful waiting but may require surgery. The outlook is usually good as long as there is no underlying pulmonary fibrosis or complications following surgery. The disease is highly uncommon. Signs and symptoms
Signs
Reduced movement of the ribcage during breathing, reduced breath sounds on the affected side(s), and a dull feeling when the chest is pressed are common signs observed during examination for fibrothorax. Sharp chest pain with deep breaths or coughing may be seen in some cases. Severe cases of fibrothorax can lead to respiratory failure due to inadequate ventilation and cause abnormally high levels of carbon dioxide in the bloodstream. Symptoms
The condition only causes symptoms if the visceral pleura is affected.