The formation of generalized permanent scar tissue in the connective tissue of the lungs, or surrounding the bronchi (see Intersitial lung disease, Diagnostic Code: 6825 Diffuse interstitial fibrosis (interstitial pneumonitis, fibrosing alveolitis).

The cause of pulmonary fibrosis is often unidentified, even though many diseases may cause the condition. Pulmonary and peribronchial fibrosis may result subsequent to inflammatory conditions, infection, immune system abnormalities, radiation, drugs, poisons, or pulmonary disease. Commonly, environmental or occupational inhalation of organic or inorganic substances, such as asbestos (see Diagnostic Code: 6833 Asbestosis) or toxic chemical fumes, can cause pulmonary fibrosis.

There may be decreased ventilatory function and altered gas exchange; shortness of breath; coughing; weight loss; fatigue; weakness; and vague chest pain. Late symptoms of pulmonary fibrosis may be cyanosis, clubbing of the fingers, and heart failure.

Pulmonary function tests, chest x-ray, computed tomography (CT) scan, bronchoscopy, lung biopsy, and laboratory studies may be done.

Corticosteroids, antibiotics, oxygen therapy, cardiac medications, and possibly lung transplantation may be included in the treatment regime.

Advanced stages of pulmonary fibrosis can lead to cor pulmonale, or may require lung transplantation. Fibrosis can occur 20 or more years after exposure to asbestos.

None.