Pulmonary fibrosis (scarring of lung due)

Chest | Pulmonology | Pulmonary fibrosis (scarring of lung due) (Disease)


Pulmonary fibrosis (scarring of the lung due) is the formation or development of excess fibrous connective tissue (fibrosis) in the lungs. It is also described as scarring of the lung. Symptoms of pulmonary fibrosis are mainly: shortness of breath, particularly with exertion, chronic dry, hacking coughing, fatigue and weakness, chest discomfort, loss of appetite and rapid weight loss.

Causes and Risk factors

Pulmonary fibrosis may be a secondary effect of other diseases. Most of these are classified as interstitial lung diseases. Examples include autoimmune disorders, viral infections or other microscopic injuries to the lung. However, pulmonary fibrosis can also appear without any known cause. In this case, it is termed idiopathic.

Diagnosis and Treatment

Most idiopathic cases are diagnosed as idiopathic pulmonary fibrosis. This is a diagnosis of exclusion of a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). In either case, there is a growing body of evidence which points to a genetic predisposition in a subset of patients. For example, a mutation in Surfactant protein C (SP-C) has been found to exist in some families with a history of pulmonary fibrosis.

There is no cure for pulmonary fibrosis. Treatment is aimed at slowing down the scarring process and preventing hypoxia (low oxygen levels). Medications such as corticosteroids and immunosuppressive medications (cyclophosphamide, azathioprine, methotrexate, penicillamine, cyclosporine) may be given to reduce the inflammation and scarring, but these treatments are not very effective. Oxygen is administered for low blood oxygen levels. ...