Idiopathic diffuse interstitial pulmonary fibrosis

Causes and Risks:
Idiopathic diffuse interstitial pulmonary fibrosis is a group of diseases of the lower respiratory tract that leads to the loss of the functional alveolar (air sac) units and a limit in the transfer of oxygen from air to blood. There is widespread inflammation and deposition of scar tissue within the lung tissue. Damage to the lung tissue occurs from a response of the immune system of unknown cause (idiopathic means "unknown cause"). The disease is more common in men than in women, and it occurs most often in people between 60 and 70 years old.

Prevention:
There is no known prevention.

Symptoms:



Signs and Tests:
Physical examination shows finger clubbing (abnormal enlargement of the tips of the fingers). Dry, crackling breath sounds may be heard by stethoscope examination of the chest.

Tests that help diagnose the disease include:



Treatment:
The objective of treatment is supportive therapy, as there are no curative treatments. Medications such as corticosteroids and cytotoxic drugs may be given to suppress the immune system.

Heart-lung and single lung transplantation may be indicated for highly selected patients with end-stage pulmonary fibrosis (a fibrotic lung disease ).

Support Groups:
The stress of illness can often be helped by joining a support group where members share common experiences and problems. See lung disease - support group .

Prognosis:
Up to 20% of patients improve with drug therapy, but the disease is progressive, with respiratory failure the eventual outcome. Average survival time is 4 years after the onset of symptoms.

Complications:



Call Your Healthcare Provider:
Call for an appointment with the health care provider if persistent cough , chest pain , and shortness of breath develop.


Spirometry is a painless study of air volume and flow rate within the lungs. Spirometry is frequently used to evaluate lung function in people with obstructive or restrictive lung diseases (such as asthma or cystic fibrosis).



This film shows advanced sarcoid, scarring of the lungs (the light streaking), and cavity formation (the dark areas in the upper right side of the picture).