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Interstitial Lung Disease
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Interstitial lung disease is a collection of pulmonary disorders that cause progressive scarring of tissue in the lungs. It makes you short of breath and not enough oxygen gets into your bloodstream. Most interstitial lung diseases have a prolonged onset but some have a rapid onset. Sometimes a reason behind the interstitial lung disease can be found and sometimes no cause can be found.
Scar tissue in the lungs can't be reversed but it can be halted in some cases. Some drugs are available to improve lung function in the presence of the scar tissue.
The various signs and symptoms of interstitial lung disease include dyspnea, with or without exertion, wheezing, a dry cough, pain in the chest and clubbing of the fingernails, a common sign of lung disease. The main symptoms of this disease are a dry cough and being short of breath. The symptoms begin mildly and become gradually worse over time. Your ability to get around without becoming short of breath decreases as time goes on.
The major finding in interstitial lung disease is scarring of the alveoli or air sacs. The sacs become inflamed and settle into a scarring pattern. The tissue that holds the air sacs together can become scarred as well. The air sacs can't expand and contract as they normally do in healthy lungs. The lungs become spongy with thick walls between the alveoli. It is felt that an abnormal healing response sets the stage for the scar tissue to form.
There are many causes of interstitial lung disease. These can include occupational exposure to toxins or pollutants or environmental exposure to a highly polluted area. Ammonia and chlorine gases are common causes of interstitial cystitis. Organic exposures, such as to grain dust, bird droppings and sugar cane dust can cause the lungs to react abnormally and can cause interstitial cystitis.
Infections with cytomegalovirus and histoplasmosis can lead to interstitial scarring as can fungal infections and parasitic infections. Radiotherapy for another reason such as cancer can cause scarring of the lung tissue. Certain drugs can cause interstitial cystitis, including drugs used for chemotherapy, drugs used for heart arrhythmias, antibiotics and some psychiatric medications. You can get interstitial lung disease if you have lupus, Sjogren's syndrome, scleroderma, rheumatoid arthritis and sarcoidosis. Even gastroesophageal reflux disease can lead to lung tissue scarring.
If no cause is found, the disease is called idiopathic pulmonary fibrosis and this is very common. Some areas of the lung are often found to be normal while other areas are filled with scar tissue. Factors that contribute to having the disease include being older than age 50, being exposed to environmental or occupational toxins, having had radiation or having had chemotherapy. High levels of oxygen inhaled for more than two days can contribute to scarring of the lungs.
Those who have idiopathic disease are more likely to be smokers, have a familial history of interstitial lung disease and have gastroesophageal reflux disease.
Complications of interstitial lung disease include having hypoxemia or low blood oxygen. You can also get high blood pressure in the lungs, called pulmonary hypertension and right sided heart failure, a condition called cor pulmonale. Eventually, you can suffer from respiratory failure and death.
It can be difficult to diagnose interstitial lung disease. Doctors can sometimes see changes on the chest x-ray or CT scan. CT scans can show interstitial lung changes better than can an x-ray. Pulmonary function tests will show whether or not there are breathing abnormalities. Exercise tests can show evidence of difficulty breathing during exercise. A bronchoscopy can be done using a biopsy tool that can show microscopic evidence of pulmonary fibrosis. A bronchoalveolar lavage is a test where saline solution is injected into the lungs and suctioned out again. Cells consistent with interstitial lung disease can be found in the lavage fluid. Thorascopic surgery can be done using a video monitor that can take biopsies from several suspicious sites in the lungs.
Doctors use steroid drugs to block some of the scar tissue of the lungs in interstitial lung disease. A drug called azathioprine is used along with corticosteroid therapy. Acetyl cysteine can act as an antioxidant and can reduce scarring. There are anti-fibrotic drugs that will reduce the development of scar tissue. Many people who have this condition need oxygen therapy to improve oxygenation. Pulmonary rehabilitation is another option. In severe cases, a lung transplant is the only life saving measure available for this disease.