What is Pulmonary Alveolar Proteinosis?

Pulmonary Alveolar Proteinosis (PAP) is a rare lung disorder. It is a lung disease in which a phospholipid (a surfactant compound that is widely distributed in living cells) abnormally accumulates within air sacks inside the lungs.

When the tiny air sacs of the lungs (alveoli) fill up with a secreted material known as surfactant, it reduces the amount of oxygen that can be diffused from the alveoli into the blood.

Secreted surfactant is helps keep the alveoli (air sacs) to remain open, however, when surfactant accumulated abnormally, it results in Pulmonary Alveolar Proteinosis lung disorder.

This rare lung disorder generally occurs in persons of 30 to 50 years of age, and it occurs in men more often than in women.

What Causes Pulmonary Alveolar Proteinosis?

In most cases, the exact cause of PAP is unknown. In others, it is often associated with potential agents such as dusts and fumes. It can also result from an infection or an immune deficiency, as indicated below:

• Primary Pulmonary Alveolar Proteinosis occurs due to:
  • Idiopathic (exact cause unknown)
  • Hereditary
• Secondary Pulmonary Alveolar Proteinosis occurs secondary to:
  • HIV infection (AIDS)
  • Lymphoproliferative disorders
  • Exposure to Silica dust,
  • Exposure to Aluminum dusy,
  • Exposure to Titanium dust,
  • Exposure to Insecticides, and
  • Tuberculosis.

Pulmonary Alveolar Proteinosis Symptoms:

In some cases, there are no symptoms. In other cases, some of the following symptoms may be present:

• Symptoms are often confused with chronic pneumonia,
• Shortness of breath (dyspnea)
• Persistent, dry cough,
• Unexplained weight loss,
• Fever, and
• Night sweats.

Pulmonary Alveolar Proteinosis Treatment:

The only treatment proven to relieve PAP symptoms is removal of the excessive surfactant material from the alveoli. The procedure to remove accumulated excessive surfactant from alveoli is known as whole lung lavage.

During this lung cleansing procedure, one lung is repeatedly washed out with a mild salt solution while the other lung is ventilated with pure oxygen. The procedure is then repeated with the other lung.

In some cases, lung transplantation is also considered.

Pulmonary Alveolar Proteinosis Prognosis:

Some people with pulmonary alveolar proteinosis suffer from shortness of breath for ever. However, this lung disorder is rarely fatal as long as the patients undergo regular lung washings.

• Many PAP patients recover on their own, not needing specific therapy,
• Some patients need whole lung lavage to relieve symptoms,
• Some patients continue to have progressive PAP disease, despite multiple lavages.
• All PAP patients are at greater risk of developing pneumonia.

Don't forget to check out Lung Disease News section of this website for the latest Pulmonary Alveolar Proteinosis / PAP Treatment News, and our Lung Disease Articles Library for the latest Pulmonary Alveolar Proteinosis (PAP) Articles.

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