Pulmonary fibrosis may be a secondary effect of other diseases, most of them being classified as interstitial lung diseases, e.g. autoimmune disorders, viral infections or other microscopic injuries to the lung. However, it may also appear without any known cause, then termed "idiopathic". Most of the idiopathic cases can be diagnosed as idiopathic pulmonary fibrosis, a diagnosis of exclusion, yet having 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.
Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include:
Inhalation of environmental and occupational pollutants,such as in asbestosis, silicosis and exposure to certain gases. Coal miners, ship workers and sand blasters among others are at higher risk.
Hypersensitivity pneumonitis, most often resulting from inhaling dust contaminated with bacterial, fungal, or animal products.
Cigarette smoking can increase the risk or make the illness worse.
Some typical connective tissue diseases such as rheumatoid arthritis, SLE and scleroderma
Other diseases that involve connective tissue, such as sarcoidosis and Wegener's granulomatosis
Certain medications, e.g. amiodarone, bleomycin, busulfan, methotrexate, and nitrofurantoin
Radiation therapy to the chest
Pulmonary fibrosis which is also called Idiopathic pulmonary fibrosis ,creates scar tissue. The scarring is permanent once it has developed.Slowing the progression and prevention depends on the underlying cause:
Treatment options for idiopathic pulmonary fibrosis are very limited.Though research trials are ongoing, there is no evidence that any medications can significantly help this condition. Lung transplantation is the only therapeutic option available in severe cases. Since some types of lung fibrosis can respond to corticosteroids (such as Prednisone) and/or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to slow the processes that lead to fibrosis.
The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Those whose conditions improve with immune suppressive treatment probably do not have idiopathic pulmonary fibrosis, for idiopathic pulmonary fibrosis has no significant treatment or cure. There are pharmacological agents in the experimental phase intended to prevent scarring. Anti-inflammatory agents have only limited success in reducing the fibrotic progress.
Some of the other types of fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may respond to immunosuppressive therapy such as corticosteroids.However, only a minority of patients respond to corticosteroids alone, so additional immunosuppressants, such as cyclophosphamide, azathioprine, methotrexate, penicillamine, and cyclosporine may be used. Colchicine has also been used with limited success. There are ongoing trials with newer drugs such as IFN-γ, mycophenolate mofetil, and pirfenidone.
Hypersensitivity pneumonitis, a less severe form of pulmonary fibrosis, is prevented from becoming aggravated by avoiding contact with the causative material.
Oxygen supplementation improves the quality of life and exercise capacity. Lung transplantation may be considered for some peolple.-
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