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Interstitial Lung Disease


Disease: Interstitial Lung Disease Interstitial Lung Disease
Category: Respiratory diseases
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Disease Definition:

In fact, Interstitial lung disease describes a group of disorders, most of which result in progressive scarring of lung tissue. This eventually affects one’s ability to breathe and get sufficient oxygen into the bloodstream. The disorders vary greatly beyond this.

When some cases of the Interstitial lung disease is abruptly appear, others mostly develop gradually. Doctors can pinpoint why some cases of interstitial lung disease occur, but many have no known cause.

In all cases, once lung scarring occurs, it’s irreversible. Medications can sometimes slow the damage of interstitial lung disease, though many people never regain full use of their lungs. Researchers hope that newer drugs, many still experimental, may finally prove more effective in treating interstitial lung disease.

Work Group:

Prepared by: Scientific Section

Symptoms, Causes


The following are signs and symptoms of the wide variety of disorders classified as interstitial lung disease:



  • Chest pain
  • Wheezing
  • A feeling of breathlessness (dyspnea), particularly throughout or after physical activity
  • Fingernails that curve over the tops of the fingertips (clubbing)
  • A dry cough

The primary signs and symptoms are usually a dry cough and breathlessness. Because these problems are subtle and tend to develop gradually — often long after the patient has irreversible lung damage — he/she may assume they are due to aging, asthma, being overweight or out of shape, smoking, or the lingering effects of an upper respiratory infection.

Symptoms seem to get progressively worse. One may finally notice they’re getting out of breath throughout routine activities like, talking on the phone, eating and even getting dressed. Breathing problems become impossible to ignore at this point.

Irreversible lung damage has often already occurred by the time signs and symptoms like cough and breathlessness appear. Nevertheless, it is important to see the doctor at the first sign of breathing problems. Several conditions other than interstitial lung disease can affect the lungs, and in order to get a prompt treatment, getting an early and accurate diagnosis is important.


Air travels to the lungs through major airways known as bronchi every time one inhales. The bronchi subdivide into smaller airways (bronchioles) inside the lungs that eventually end in clusters of tiny air sacs (alveoli). Within the walls of the air sacs are small blood vessels (capillaries), where oxygen is added to the blood and carbon dioxide (a waste product of metabolism) is removed.

Scarring of tiny air sacs
The walls of the air sacs may become inflamed in interstitial lung disease, and the tissue (interstitium) that lines and supports the sacs becomes highly thickened and scarred. The air sacs are normally highly elastic, expanding and contracting like small balloons with each breath. But scarring (fibrosis) results in stiffening and thickening of the thin, interstitial tissue making the air sacs less flexible. Scarred air sacs have the texture of a stiff sponge rather than being soft and elastic, which makes it harder for one to breathe and for oxygen to enter the bloodstream through the thickened walls.

Abnormal healing response
When an injury to the lungs triggers an abnormal healing response, scarring appears to be occurring in interstitial lung disease. The body generates just the right amount of tissue to repair damage under ordinary circumstances. But the repair process in such a condition goes awry, producing extra scar tissue that highly interferes with lung function.

One disorder, many causes
It can be hard to determine the reason for an initial injury to lung tissue because interstitial lung disease has a wide range of causes. The following are some of several possible leading aspects:

Occupational and Environmental Factors:
Long-term exposure to a number of toxins or pollutants can contribute to serious lung damage. Workers who routinely inhale silica dust, asbestos fibers or hard metal dust are particularly at risk of serious lung disease. So are people exposed to some chemical fumes and ammonia or chlorine gases.
The lungs can be damaged as well by the chronic exposure to a wide range of substances, many of them being organic. Sugar cane, animal droppings, grain and dust from bird are among these. Other substances, like moldy hay, can be a problem when they result in a hypersensitivity reaction in the lungs (hypersensitivity pneumonitis). Even bacterial or fungal overgrowth in poorly maintained humidifiers and hot tubs can result in lung damage.

Infections:  They include bacterial infections like cytomegalovirus, a particular problem when having a weakened immune system, pneumonia from bacterial infections, parasitic infections and fungal infections like histoplasmosis.
Radiation: Signs of lung damage appear long after finishing radiation treatment in certain people who receive radiation therapy for lung or breast cancer. The severity of the damage may depend on how much of the lung is exposed to radiation, the total amount of radiation one receive, whether chemotherapy is used as well, and whether one has underlying lung disease.
Drugs: Certain drugs can damage the tissue that lines the lungs. Those most likely to result in lung problems such as chemotherapy drugs, other cardiovascular problems and medications used to treat heart arrhythmias, some antibiotics and some psychiatric medications.
Other Medical Conditions: Interstitial lung disease can occur with other disorders. Those conditions usually don’t directly attack the lungs, but rather affect tissue processes throughout the body. Scleroderma, dermatomyositis, polymyositis, sarcoidosis, Sjogren’s syndrome, lupus and rheumatoid arthritis are among these conditions. Some researchers think that gastroesophageal reflux disease (GERD), in which the lungs chronically intake a small volume of gastric contents, can cause pulmonary fibrosis.

Unknown cause
Mostly it isn’t known why exactly some people develop interstitial lung disease, even though doctors may determine why some people experience interstitial lung disease. Disorders without a known reason are grouped together under the label idiopathic pulmonary fibrosis or idiopathic interstitial lung disease. Although the idiopathic diseases have certain features in common, each also has unique characteristics.

Idiopathic pulmonary fibrosis, often known as interstitial pneumonitis, is the most common of the idiopathic interstitial lung diseases. Some areas of the lungs are normal when others are inflamed and still others are marked by scar tissue due to the developing of the usual interstitial pneumonitis in patches. The disease affects more men than women and often develops in people over age 50.
The following are other less common types of idiopathic pulmonary fibrosis:



  • Lymphocytic interstitial pneumonitis
  • Respiratory bronchiolitis-associated interstitial lung disease
  • Nonspecific interstitial pneumonitis
  • Acute interstitial pneumonitis
  • Bronchiolitis obliterans with organizing pneumonia (BOOP)
  • Desquamative interstitial pneumonitis

The following are the factors that may make one more susceptible to interstitial lung disease:

Age: exposure to occupational and environmental toxins, radiation and chemotherapy.
Oxygen: Lungs can be harmed due to continually inhaling very high levels of therapeutic oxygen for more than 48 hours.

Risk factors for idiopathic interstitial lung disease
Some factors, which have been identified by researchers, appear to be increasing the risk of idiopathic lung disease, although it isn’t known why the disorder has occurred:

Smoking: Some forms of interstitial lung disease are more likely to occur in people with a history of smoking, and active smoking may worsen the condition. The risk appears to increase with the number of years and the number of cigarettes smoked. Yet, with rare exceptions, smoking does not directly result in interstitial lung disease.
Genetic factors: Familial pulmonary fibrosis is a rare kind of idiopathic interstitial lung disease that runs in families; it’s similar to other kinds of the disease. Although research is being done on familial pulmonary fibrosis, researchers haven't yet identified the genes that may be involved.
Gastroesophageal reflux disease (GERD): Researchers are investigating a possible link between idiopathic interstitial lung disease and gastroesophageal reflux disease. In GERD, stomach acid or, sometimes, bile salts back up into the esophagus and are then drawn into the lungs (aspirated) in droplets.



Scar tissue formation in the lungs can lead to a series of life-threatening complications, such as:

Respiratory failure: This condition occurs when severely low blood oxygen levels along with rising pressures in the pulmonary arteries result in heart failure, in the end stage of chronic interstitial lung disease.
Low blood oxygen levels (hypoxemia): Since interstitial lung disease decreases the amount of oxygen one takes in and the amount that enters the bloodstream, people likely to develop lower than normal blood oxygen levels. Lack of oxygen can severely disrupt the body’s basic functioning.
Right-sided heart failure (cor pulmonale): As the heart’s lower right chamber (right ventricle) which is less muscular than the left, feels the urge to pump harder than usual to move blood through obstructed pulmonary arteries, the serious condition of right-sided heart failure occurs. The right ventricle eventually fails from the extra strain.

High blood pressure in your lungs (pulmonary hypertension): Pulmonary hypertension is a serious illness that becomes progressively worse. This condition affects only the arteries in the lungs, unlike systemic elevated blood pressure. It starts when scar tissue restricts the smallest blood vessels, limiting blood flow in the lungs. This in turn increases pressure within the pulmonary arteries.


Toxins or drugs that cause interstitial lung disease can sometimes be reversed when they’re no longer exposed to those substances. But in people for whom this isn’t the case, the outlook is less promising. Because of the serious side effects of the presently available drug therapies, they aren’t usually working best.

A combination of the following medications are usually included among the treatment for interstitial lung disease:

Corticosteroid drugs: These anti-inflammatory drugs help a minority of people with interstitial lung disease. Those most likely to benefit have a disorder with a known cause and reversible changes in their lungs. Corticosteroids rarely improve lung function in people suffering from idiopathic pulmonary fibrosis, and when they do, the benefits are usually temporary.
Corticosteroids can cause a number of side effects when either taken in large doses, or for long period of time, such as bone loss, elevated blood sugar levels contributing to diabetes, glaucoma, high susceptibility to infection and poor wound healing.

Azathioprine: This cytotoxic medication that is under normal circumstances used to prevent organ rejection after a transplant, may additionally be used in combination with corticosteroids to treat interstitial lung disease. Azathioprine can result in severe side effects, such as reduced production of red blood cells and a high risk of infection and certain cancers. In case azathioprine is not effective, another cytotoxic drug, cyclophosphamide may be tried. Yet, the side effects related to cyclophosphamide are even more severe.

Acetylcysteine: This medication belongs to a class of drugs known as antioxidants. Oxidation is a natural process that contributes to cell and tissue damage. The process may play a role in the development of scarring in the lungs (pulmonary fibrosis). The outcome of a clinical trial when combined with corticosteroids and acetylcysteine, azathioprine improved lung function in people suffering from idiopathic pulmonary fibrosis. There weren’t any significant changes noted in mortality rates.

Anti-fibrotics: These drugs are sometimes used to help decrease the development of scar tissue. The medications bosentan and pirfenidone have indicated in clinical studies promise for slowing the progression of lung damage. Follow-up studies to gain more information about the risks and benefits of these drugs in the treatment of interstitial lung disease are under way. Other anti-fibrotic agents that have been studied as possible treatments for this condition, including colchicines, interferon gamma-1b and penicillamine have not been found effective.

Oxygen therapy: The doctor may recommend oxygen therapy depending on one’s activity level and the severity of symptoms. It can make breathing and exercise easier and prevent or lessen complications from low blood oxygen levels, even though oxygen can't stop lung damage. Oxygen therapy may additionally improve the sleep and sense of well-being. It can decrease blood pressure in the right side of the heart as well.

Pulmonary rehabilitation: Pulmonary rehabilitation is a formal program for people suffering from chronic lung disease that includes, but goes far beyond, medical management. The aim of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning, but to help people suffering from pulmonary fibrosis live full, satisfying lives as well. These programs concentrate on exercise, teaching one how to breathe more efficiently, emotional support, nutritional counseling and education.
This multifaceted approach needs most frequently a team of health care providers that may include a doctor, rehabilitation specialist, social worker, dietitian and nurse. However, programs vary widely. The doctor can often tell one about pulmonary rehabilitation programs in the area.

Lung transplantation: This may be an option for people suffering from severe interstitial lung disease who are unable to benefit from other treatment options.

One should agree to quit smoking in case they are smokers, be sufficiently healthy to undergo surgery and post-transplant treatments, be willing and able to follow the medical program outlined by the rehabilitation and transplant team and have the patient and emotional strength and support to undergo the wait for a donor organ in order to be considered for a transplant. Support to undergo the wait for a donor organ is especially important due to donor organs being in short supply.


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