Pulmonary fibrosis

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Pulmonary fibrosis is a irreversible and progressive disease in which the air sacs of the lungs become stiff and hard to fill, making breathing increasingly more difficult. It is a member of the class of interstitial lung diseases (ILD).

Etiology

A number of forms of the disease come from industrial exposure to coal dust or sand blasting; other cases are due to drug reactions, and sometimes the cause is never known (i.e., idiopathic pulmonary fibrosis). It may be secondary to other diseases such as scleroderma or rheumatoid arthritis.[1] Familial pulmonary fibrosis is inherited. [2]

Pathology

Formally, it is a "process in which normal lung tissues are progressively replaced by fibroblasts and collagen causing an irreversible loss of the ability to transfer oxygen into the bloodstream via pulmonary alveoli. Patients show progressive dyspnea finally resulting in death."[3]

Treatment

There is no single approved treatment, although there are many supportive measures, both pharmacologic and nonpharmacologic. Pulmonary fibrosis is the most common indication for lung transplantation, which is not without risk.

References