Some types of these diseases are much more serious than others.
Diagnosis requires chest x-rays, computed tomography, and usually analysis of a sample of lung tissue (biopsy).
Treatment depends on the disease type.
(See also Overview of Interstitial Lung Diseases Overview of Interstitial Lung Diseases Interstitial lung disease (also called diffuse parenchymal disease) is a term used to describe a number of different disorders that affect the interstitial space. The interstitial space consists... read more .)
The word idiopathic means of unknown cause, so when the cause of interstitial lung disease is not identified, idiopathic interstitial pneumonia is diagnosed. Pneumonias are often thought of as infections, but these diseases do not appear to result from infection.
There are eight types of idiopathic interstitial pneumonias. In decreasing order of frequency, they are
Desquamative interstitial pneumonia Respiratory Bronchiolitis–Associated Interstitial Lung Disease and Desquamative Interstitial Pneumonia Respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia are rare conditions that cause chronic lung inflammation and occur mostly in current or... read more
Respiratory bronchiolitis-associated interstitial lung disease Respiratory Bronchiolitis–Associated Interstitial Lung Disease and Desquamative Interstitial Pneumonia Respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia are rare conditions that cause chronic lung inflammation and occur mostly in current or... read more
All types cause cough and shortness of breath and affect the lungs similarly. The ends of the fingers may become thick or club-shaped (see figure Recognizing Finger Clubbing Recognizing Finger Clubbing Clubbing is enlargement of the tips of the fingers or toes and a change in the angle where the nails emerge. Clubbing occurs when the amount of soft tissue beneath the nail beds increases. It... read more 
). Through a stethoscope, doctors often hear crackling sounds. Although symptoms may be similar, disorders differ in how quickly symptoms develop, how they are treated, and how serious they are.
Diagnosis of Idiopathic Interstitial Pneumonias
Chest computed tomography
Computed tomography (CT) of the chest is the main imaging tool used in the evaluation of idiopathic interstitial pneumonias. CT may allow doctors to make a diagnosis. If not, doctors remove a small sample of lung tissue for examination under a microscope (lung biopsy). Usually, biopsy is done surgically with use of a thoracoscope Thoracoscopy Thoracoscopy is the visual examination of the lung surfaces and pleural space through a viewing tube (a thoracoscope). Thoracoscopy is used to view the lung and the space surrounding the lungs... read more .
Blood tests are usually done. They usually cannot confirm the diagnosis but are done as part of the search for other disorders. Doctors may also do electrocardiography Electrocardiography Electrocardiography (ECG) is a quick, simple, painless procedure in which the heart’s electrical impulses are amplified and recorded. This record, the electrocardiogram (also known as an ECG)... read more 
(ECG) or echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more 
to determine whether the heart has been affected by the lung disease.
Treatment of Idiopathic Interstitial Pneumonias
Smoking cessation
Often drugs, corticosteroids or novel antifibrotic drugs, depending on the disease
Sometimes lung transplantation
Smoking cessation Smoking Cessation While often very challenging, quitting smoking is one of the most important things smokers can do for their health. Quitting smoking brings immediate health benefits that increase over time... read more is always recommended because smoking is likely to make the disorder progress more rapidly.
Other treatments depend on the type of idiopathic interstitial pneumonia but may include corticosteroids or other drugs, such as novel antifibrotic drugs.
Drugs Mentioned In This Article
| Generic Name | Select Brand Names |
|---|---|
Pirfenidone |
ESBRIET |
nintedanib |
OFEV |
