What is Alveolar Proteinosis?

Alveolar proteinosis is a serious condition that generally has no identified cause, though a few people with silicosis or infection develop it. It affects the lungs or more accurately the alveoli. These are sacs of air within the lungs and they can be filled with protein build-up, reducing their capacity and creating great difficulty. While, in most circumstances, alveolar proteinosis doesn’t have a known cause, it is known that the condition is most likely to affect those slightly above or below the age of 40, and treatment for the disease can be variable depending on degree of symptoms expressed.

More accurately titled pulmonary alveolar proteinosis (PAP), this disease may have a few initial symptoms that might be noticed. These include trouble breathing or dyspnoea. Different forms of this can include shortness of breath or labored breathing.

While dyspnoea is common when people exercise strenuously, with PAP it might occur in a resting state or after mild exertion. Other symptoms of this illness could include a cough that may or may not produce mucus and elevated body temperature. Some people feel very tired due to poor breathing and less oxygenation, and others will suddenly drop weight without effort. To confuse matters, though, in early stages people might not have any symptoms of all, and diagnosis of the illness would be unlikely.

When alveolar proteinosis is suspected, there can be several tests done to confirm 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 examine protein levels in the lungs and may be useful in confirming presence of the disease. Sometimes other tests are needed too, including biopsy of the lung.

There are a number of factors that may determine treatment of alveolar proteinosis. The present condition of the person affected makes a difference. When people have no symptoms or aren’t severely inconvenienced, they might not have any treatment. Also, in about one in ten cases, PAP goes away on its own within a year, and may not return.

Many people do need some form of treatment, and the most common medical response is to do lavage, rinsing out, of one or both lungs. This has been shown to effective for many people, reducing symptoms for a long time. Repetition of lavage could be required if 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 a worse case scenario, alveolar proteinosis becomes so severe that lavage is not working. Under these circumstances, one option available to patients may be lung transplant. This option is rarely exercised because the same condition and protein build up is likely to occur in the transplanted lung. People with this illness could rank low on any transplant lists, even if they are in dire need of a new lung and have a doctor supporting this treatment.