Although fibrothorax may not cause any symptoms, people affected by the disorder may report shortness of breath. Persistent, recurrent pleural effusions are a possible symptom, caused by the persistent cavity formed by the hardening pleura around the original fluid collection. Shortness of breath tends to develop gradually and may worsen over time. Less commonly, fibrothorax may cause chest discomfort or a dry cough. Fibrothorax may occur as a complication of other diseases. Symptoms of the underlying problem are sometimes seen, for example, fever in cases of empyema. Causes
Fibrothorax is often a complication of other diseases that cause inflammation of the pleura. These include infections such as an empyema or tuberculosis, or bleeding within the pleural space known as a haemothorax. Exposure to certain substances, such as asbestos, can cause generalised fibrosis of the lungs, which may involve the pleura and lead to fibrothorax. Less common causes of fibrothorax include collagen vascular diseases such as systemic lupus erythematosus, sarcoidosis, and rheumatoid arthritis; kidney failure leading to uraemia; and side effects of certain medications. The medications most commonly associated with pleural fibrosis are the ergot alkaloids bromocriptine, pergolide, and methysergide. Fibrothorax may also occur without a clear underlying cause, in which case it is known as idiopathic fibrothorax. A technique called pleurodesis can be used to intentionally create scar tissue within the pleural space, usually as a treatment for repeated episodes of a punctured lung, known as a pneumothorax, or for pleural effusions caused by cancer.
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.