Diffuse interstitial pulmonary fibrosis

Causes and Risks:
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. There are approximately 180 different disorders within this disease classification and are further categorized by known and unknown causes. The known causes of diffuse interstitial pulmonary fibrosis are numerous and include inorganic and organic dusts, gases, fumes, vapors, medications, radiation, and certain lung infections. Hypersensitivity pneumonitis , coal worker's pneumoconiosis , silicosis , and byssinosis (cotton dust) are some of the occupational lung diseases that can lead to diffuse interstitial pulmonary fibrosis. The cause may also be unknown ( idiopathic diffuse interstitial pulmonary fibrosis ). Risk factors are related to the known causes of these disorders. Smoking increases the risk. People over 40 years old are affected more frequently. The incidence is 1 out of 1000 people.

Prevention:
Avoid exposure to agents known to cause lung disease .

Symptoms:

Additional symptoms that may be associated with this disease:



Signs and Tests:



Treatment:
The treatment depends upon the underlying cause of the disease. If there is no specific treatment for an underlying disorder, supportive therapy is indicated.

Exposure to agents known to cause lung disease (inorganic and organic dusts, gases, fumes, vapors, medications, and radiation) should be restricted. Heart-lung and single-lung transplantation may be indicated for highly selected patients with end-stage pulmonary fibrosis .

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

Prognosis:
Recovery or disease progression varies with the underlying cause of the condition. In general, it is a progressive disease.

Complications:



Call Your Healthcare Provider:
Call the health care provider if you have shortness of breath at rest that lasts for any duration.


Clubbing results from chronic low blood-oxygen levels. This can be seen with cystic fibrosis, congenital cyanotic heart disease, and several other diseases. The tips of the fingers enlarge and the nails become extremely curved from front to back.



This chest X-ray shows coal workers pneumoconiosis - stage II. There are diffuse, small (2 to 4 mm) light areas on both sides of the lungs. Diseases which may explain these X-ray findings include simple coal workers pneumoconiosis (CWP) - stage II, simple silicosis, disseminated tuberculosis, metastatic lung cancer, and other diffuse infiltrative pulmonary diseases.



This chest X-ray shows coal workers pneumoconiosis - stage II. There are diffuse, small (2 to 4 mm each), light areas throughout both lungs. In the right upper lung (seen on the left side of the picture), there is a light area (measuring approximately 2 cm by 4 cm) with poorly defined borders, representing coalescence (merging together) of previously distinct light areas. Diseases which may explain these X-ray findings include simple coal workers pneumoconiosis (CWP) - stage II, silico-tuberculosis, disseminated tuberculosis, metastatic lung cancer, and other diffuse infiltrative pulmonary diseases.



This picture shows complicated coal workers pneumoconiosis. There are diffuse, small, light areas (3 to 5 mm) in all areas on both sides of the lungs. There are large light areas which run together with poorly defined borders in the upper areas on both sides of the lungs. Diseases which may explain these X-ray findings include complicated coal workers pneumoconiosis (CWP), silico-tuberculosis, disseminated tuberculosis, metastatic lung cancer, and other diffuse infiltrative pulmonary diseases.