[3] Severe cases of fibrothorax can lead to respiratory failure due to inadequate ventilation and cause abnormally high levels of carbon dioxide in the bloodstream.

Exposure to certain substances, such as asbestos, can cause generalised fibrosis of the lungs, which may involve the pleura and lead to fibrothorax.

[7] 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 term fibrothorax implies severe fibrosis affecting both the visceral and the outer (parietal) pleura, fusing the lung to the chest wall.

[4] Over time, generally over the years, the fibrotic scar tissue slowly tightens and thickens, contracting the contents of one or both halves of the chest and reducing the mobility of the ribs.

[7] Restrictive lung disease from fibrothorax may occur when pleural fibrosis is so severe that it involves the diaphragm and ribcage and results primarily from decreased rib movement.

[3] At the microscopic level, collagen fibres deposit in a basket weave pattern and form scar tissue.

However, research indicates a protein called Transforming Growth Factor beta (TGF-β) plays a central role in producing fibrothorax.

[3] 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.

[6] Conservative non-surgical treatment of fibrothorax is generally done by treating its underlying cause and is reserved for milder cases.

[9] Severe cases of fibrothorax may require supportive mechanical ventilation if the affected person is unable to breathe adequately on their own.

[3] Surgical decortication is generally considered for people with fibrothoraces that are severe, causing significant shortness of breath, and have otherwise relatively healthy lungs since this enhances the likelihood of a better outcome.

[10] Fibrothorax complicating another condition, such as tuberculous pleuritis, empyema, or acute haemothorax often spontaneously resolves in 3-6 months.

[3] The prognosis after surgical decortication is variable and depends on the health of the underlying lung before the procedure takes place.

[10] Sporadic cases are rarely reported in the medical literature, for example, due to iatrogenic or postoperative complications.