Interstitial Lung Diseases (Pulmonary Fibrosis)
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Interstitial lung disease, or ILD, includes more than 180 chronic lung disorders, which may be:
•chronic
•nonmalignant (non-cancerous)
•noninfectious
Interstitial lung diseases are named for the tissue between the air sacs of the lungs called the interstitium -- the tissue affected by fibrosis (scarring). Its also called interstitial pulmonary fibrosis or pulmonary fibrosis.
The symptoms and course of these diseases may vary from person to person but all begin with Inflamation.
•bronchiolitis - inflammation that involves the bronchioles (small airways)
•alveolitis - inflammation that involves the alveoli (air sacs)
•vasculitis - inflammation that involves the small blood vessels (capillaries)
More than 80 percent of interstitial lung diseases are diagnosed as pneumoconiosis, a drug-induced disease, or hypersensitivity pneumonitis. The other types are:
•sarcoidosis
•idiopathic pulmonary fibrosis
•bronchiolitis obliterans
•histiocytosis X
•chronic eosinophilic pneumonia
•collagen vascular disease
•granulomatous vasculitis
•Goodpasture's syndrome
•pulmonary alveolar proteinosis
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In interstitial lung disease, the lung is affected in three ways:
1.Lung tissue is damaged in some known or unknown way.
2.The walls of the air sacs in the lungs become inflamed.
3.Scarring (fibrosis) begins in the interstitium.
Fibrosis results in permanent loss of that tissue's ability to breathe and carry oxygen. Air sacs, as well as the lung tissue between and surrounding the air sacs, and the lung capillaries, are destroyed by the formation of scar tissue.
Symptoms of interstitial lung diseases:
The following are the most common symptoms for interstitial lung diseases; however, each person may experience symptoms differently. Symptoms may include:
•shortness of breath, especially with exertion
•fatigue and weakness
•loss of appetite
•loss of weight
•dry cough that does not produce phlegm
•discomfort in chest
•labored breathing
•hemorrhage in lungs
The symptoms of interstitial lung diseases may resemble other lung conditions or medical problems. Consult your doctor for a diagnosis.
CAUSES OF INTERSTITIAL LUNGS DISEASE:
The cause of interstitial lung disease is not known; however, a major contributing factor is thought to be inhaling environmental pollutants. Other contributing factors include:
•sarcoidosis
•certain drugs or medications
•radiation
•connective tissue or collagen diseases
•family history
DIAGNOSIS
In addition to a complete medical history and physical examination, the doctor may also request the following tests:
•pulmonary function tests - to determine characteristics and capabilities of the lungs
◦spirometry - to measure the amount of air that can be forced out
◦peak flow meter - to evaluate changes in breathing and response to medications
•blood tests - to analyze the amount of carbon dioxide and oxygen in the blood
•x-ray
•computerized axial tomography (CAT) scan
•bronchoscopy - to examine the lung using a long, narrow tube called a bronchoscope
•bronchoalveolar lavage - to remove cells from lower respiratory tract to help identify inflammation and exclude certain causes
•lung biopsy - to remove tissue from the lung for examination in the pathology laboratory
Treatment:
Specific treatment will be determined by your doctor based on:
•your age, overall health and medical history
•extent of the disease
•your tolerance for specific medications, procedures or therapies
•expectations for the course of the disease
•your opinion or preference
Treatments may include:
•oral medications, including corticosteroids
•influenza vaccine
•pneumococcal pneumonia vaccine
•oxygen therapy from portable tanks
•lung transplantation
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