Interstitial Lung Disease and Pulmonary Fibrosis

Interstitial Lung Disease and Pulmonary Fibrosis are general terms used to describe inflammatory and fibrotic disorders of lung tissue (interstitium). There are over 100 known causes of interstitial lung disease and pulmonary fibrosis, which include familial and genetic disorders, inhaled substances, infections, medications, and connective tissue diseases.

Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by chronic inflammation, accompanied by an uncontrolled healing response that causes progressive scarring or thickening (fibrosis) of tissues between the lung's alveoli, or air sacs. The cause of IPF is unknown, although the body's own immune response seems to play a major role. Recent data suggest approximately 28 cases per 100,000 population. The usual age at diagnosis is between 40 and 70, but often there is X-ray evidence of disease two to five years before the diagnosis is made. The disease affects more men than women.

Patients usually present with a subtle onset of breathlessness with exercise. Over time there is a progressive worsening of breathlessness, as oxygen transfer to the blood decreases. Heart failure often develops. The median survival for people with IPF is five years.

The diagnosis of interstitial lung disease has become significantly more accurate as a result of thoracoscopy, a minimal access procedure that uses small incisions and video-endoscopic instruments to view the chest. Multiple areas of the lung can be biopsied and scanned to determine the presence of this disorder without the need for a large incision.

Standard treatment for IPF is intended to improve symptoms and slow progression of the disease. Corticosteroids and cytotoxic drugs aim to reduce the inflammatory reaction and prevent the scarring and thickening of lung tissues. A new FDA approved drug to treat pulmonary fibrosis will soon be available, providing an additional treatment option for patients with this disease. Lung transplantation has been successful as a treatment of last resort.

Click here to view a Webinar on Lung Transplantation for Pulmonary Fibrosis.

Additional information about IPF can be found a NLM/NIH Medline Plus.