What is the definition of Pulmonary Alveolar Proteinosis?

Pulmonary alveolar proteinosis (PAP) is a rare disease in which a type of protein builds up in the air sacs (alveoli) of the lungs, making breathing difficult. Pulmonary means related to the lungs.

What are the alternative names for Pulmonary Alveolar Proteinosis?

PAP; Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis; Alveolar lipoproteinosis phospholipidosis

What are the causes for Pulmonary Alveolar Proteinosis?

In some cases, the cause of PAP is unknown. In others, it occurs with lung infection or an immune problem. It also can occur with cancers of the blood system, and after exposure to high levels of environmental substances, such as silica or aluminum dust.

People between 30 and 50 years old are most often affected. PAP is seen in men more often than in women. A form of the disorder is present at birth (congenital).

What are the symptoms for Pulmonary Alveolar Proteinosis?

Symptoms of PAP may include any of the following:

  • Shortness of breath
  • Cough
  • Fatigue
  • Fever, if there is lung infection
  • Bluish skin (cyanosis) in severe cases
  • Weight loss

Sometimes, there are no symptoms.

What are the current treatments for Pulmonary Alveolar Proteinosis?

Treatment involves washing out the protein substance from the lung (whole-lung lavage) from time to time. Some people may need a lung transplant. Avoiding dusts that might have caused the condition is also recommended.

Another treatment that may be tried is a blood-stimulating drug called granulocyte-macrophage colony stimulating factor (GM-CSF), which is lacking in some people with alveolar proteinosis.

What are the support groups for Pulmonary Alveolar Proteinosis?

More information and support for people with pulmonary alveolar proteinosis and their families can be found at:

  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
  • PAP Foundation -- www.papfoundation.org/

What is the outlook (prognosis) for Pulmonary Alveolar Proteinosis?

Some people with PAP go into remission. Others have a decline in lung function (respiratory failure) that gets worse, and they may need a lung transplant.

When should I contact a medical professional for Pulmonary Alveolar Proteinosis?

Call your provider if you develop serious breathing symptoms. Shortness of breath that gets worse over time may signal that your condition is developing into a medical emergency.

Respiratory system

REFERENCES

Levine SM. Alveolar filling disorders. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 85.

Trapnell BC, Mccarthy C. Pulmonary alveolar proteinosis syndrome. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 98.

  • Condition: Pulmonary Alveolar Proteinosis
  • Journal: BMC pulmonary medicine
  • Treatment Used: One-Session Bilateral Sequential Whole Lung Lavage
  • Number of Patients: 13
  • Published —
The study researched the effects of a one-session bilateral sequential whole lung lavage (OSBSWLL) for the management of pulmonary alveolar proteinosis.