What’s alveolar proteinosis?

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Alveolar proteinosis is a serious condition that usually has no identified cause, although some people with silicosis or infection develop it. It affects the lungs or more precisely the alveoli. These are air sacs within the lungs and can become filled with protein buildup, reducing their capacity and creating great difficulty. While, in most cases, alveolar proteinosis has no known cause, the condition is known to be more likely to affect those slightly over or under the age of 40, and treatment for the disease can vary depending on the degree of symptoms expressed.

More accurately referred to as pulmonary alveolar proteinosis (PAP), this disease can have some initial symptoms that may be noticed. These include trouble breathing or wheezing. Different forms of this can include shortness of breath or wheezing.

While dyspnea is common when people exercise strenuously, with PAP it could occur in a state of rest or after mild exertion. Other symptoms of this disease might include a cough that may or may not produce mucus and a high body temperature. Some people feel very tired due to poor breathing and less oxygen, while others suddenly lose weight without effort. To confuse matters however, in the early stages people may not have any symptoms at all and the diagnosis of the disease would be unlikely.

When alveolar proteinosis is suspected, several tests may be done to confirm the diagnosis. These include x-ray and bronchoalveolar lavage. The latter puts fluid into parts of the lungs via a bronchoscope inserted into the mouth. The test can look at protein levels in the lungs and can be useful in confirming the presence of the disease. Sometimes other tests are needed as well, including a lung biopsy.

There are a number of factors that can determine the treatment of alveolar proteinosis. The current condition of the affected person makes a difference. When people have no symptoms or are not severely bothered, they may not receive any treatment. Also, in about one in ten cases, the PAP goes away on its own within a year and may not come back.

Many people need some form of treatment and the most common medical response is to flush one or both lungs. This has proven effective for many people, reducing symptoms for a long time. It may be necessary to repeat the wash if the symptoms increase again, as they often do. People with alveolar proteinosis are also prone to certain opportunistic infections of the lungs and may occasionally need antibiotic or antifungal treatment.

In the worst case, alveolar proteinosis becomes so severe that lavage doesn’t work. In these circumstances, one option available to patients may be lung transplantation. This option is rarely used because the same condition and protein buildup are likely to occur in the transplanted lung. People with this disease might rank low on any transplant list, even if they are in desperate need of a new lung and have a doctor who supports this treatment.

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