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Interstitial lung disease is a type of lung condition that results from inflammation of the interstitium or tissue that surrounds and separates the air sacs or alveolae of lungs. This supportive tissue has no purpose in the exchange of oxygen and carbon dioxide but it affects the elasticity of the lung tissue. Interstitial inflammation generally involves the entire lung at once and not just a single area.

The term "interstitial pneumonia" is a word used to describe some forms of interstitial lung disease. If interstitial disease is because of an interstitial pneumonia, it is related to some kind of infection the person came down with that caused the interstitial lung disease in the first place. Some types of interstitial lung disease are unrelated to an infection. In such cases, the lung disease is called "interstitial pneumonitis".

The causes of interstitial lung disease are often related to an immune problem in the lungs that cause an overreactive immune system. Causes can include lung infections, toxins or chemicals in the environment, including silica dust and asbestos exposure, certain medications, such as medications used for chemotherapy, radiotherapy to the lungs due to cancer, and chronic autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus and scleroderma.

In some cases, no specific cause of the lung disease can be found and the condition is then called idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has several different subdivisions, including usual interstitial pneumonitis, lymphocytic interstitial pneumonitis, bronchiolitis obliterans, and desquamative interstitial pneumonitis. These look different under the microscope but have similar symptoms and all have no known cause.

The symptoms of interstitial lung disease are related to the inability of the lungs to expand and contract properly. There is scar tissue and thickening of the supportive tissue of the lungs. Breathing is insufficient and there are reduced levels of oxygenation in the bloodstream. The most common symptoms are shortness of breath and a dry cough. Other symptoms or signs include a reduced level of oxygen in the pulse oximetry testing, clubbing of the fingers and an enlarged heart. One can get pulmonary hypertension or high blood pressure in the pulmonary blood vessels and this can result in right heart failure or cor pulmonale. After having the condition for a long time, one can get respiratory failure and death.

Doctors diagnose interstitial lung disease by getting a complete history and physical examination that looks at the lungs specifically. Breathing tests of the lungs are often abnormal, including spirometry testing. Blood tests can check for autoimmune diseases as a possible cause of interstitial lung disease. CT scan or chest x-rays of the lung may show abnormalities consistent with interstitial lung disease and can rule out other diseases. Pulmonary function testing is a good test for lung disease and often show poor outflow of air in the lungs on spirometry testing.

A biopsy of the lungs may need to be done to confirm the diagnosis. This is often done via a bronchoscope and takes a sampling of lung tissue for microscopic use. An open lung biopsy can also be done if a large sample of tissue is needed of the lungs.

The treatment of interstitial lung disease can be difficult, depending on the cause. Corticosteroid drugs are used to decrease the inflammation of the lungs and are used in inhaled forms or in oral forms. Steroids can be used for an indefinite period of time or episodically. Drugs that suppress the immune system, including azathioprine or cyclophosphamide, are used to block the immune system from causing scarring in the lung tissue. Some patients can use both forms of therapy together. It is important to quit smoking and to avoid exposure to those things that make the lung condition worse such as environmental exposures.

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Medical Negligence Solicitors

Our personal injury solicitors operate a specialist medical negligence compensation service. Our Interstitial Lung Disease solicitors deal with claims using a no win no fee arrangement which means that if you don�t win then you don�t pay them their professional costs. If you would like legal advice at no cost with no further obligation just complete the contact form or email our lawyers offices or use the helpline and a Interstitial Lung Disease solicitor will review your medical negligence compensation claim and phone you immediately.

HELPLINE: ☎ 1800 633 634

The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here