Learn About 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.
PAP; Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis; Alveolar lipoproteinosis phospholipidosis
In some cases, the cause of PAP is unknown. In others, it occurs with a 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).
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.
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 dust that might have caused the condition is also recommended.
Another treatment that may be tried is a blood-stimulating medicine called granulocyte-macrophage colony stimulating factor (GM-CSF), which is lacking in some people with alveolar proteinosis.
Matthias Griese practices in Munich, Germany. Mr. Griese is rated as an Elite expert by MediFind in the treatment of Pulmonary Alveolar Proteinosis. His top areas of expertise are Pulmonary Alveolar Proteinosis, Lacrimo-Auriculo-Dento-Digital Syndrome, Acute Interstitial Pneumonia, Interstitial Lung Disease, and Lung Transplant.
Etsuro Yamaguchi practices in Nagakute, Japan. Yamaguchi is rated as an Elite expert by MediFind in the treatment of Pulmonary Alveolar Proteinosis. Their top areas of expertise are Pulmonary Alveolar Proteinosis, Sarcoidosis, Rheumatoid Lung Disease, and Acute Interstitial Pneumonia.
Vincent Cottin practices in Lyon, France. Mr. Cottin is rated as an Elite expert by MediFind in the treatment of Pulmonary Alveolar Proteinosis. His top areas of expertise are Pulmonary Fibrosis, Interstitial Lung Disease, Acute Interstitial Pneumonia, Lung Transplant, and Angioplasty.
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 -- papfoundation.org/
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.
Contact 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.
Summary: The major goal of this study is to evaluate a new type of cell transplantation therapy for individuals with hereditary PAP, study a new treatment that may be useful for treatment of other diseases, and research mechanisms that drive the development and function of lung macrophages.
Summary: The major goal of Part A of this study is to establish a National PAP Registry to help make reliable new research tests available to doctors to improve the diagnosis of PAP, increase awareness and knowledge of PAP, and give patients a 'seat at the table' in planning and conducting PAP research including the clinical testing of several new potential therapies. The major goal of Part B of this study...
Published Date: April 10, 2025
Published By: Allen J. Blaivas, DO, Division of Pulmonary, Critical Care, and Sleep Medicine, VA New Jersey Health Care System, Clinical Assistant Professor, Rutgers New Jersey Medical School, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Levine SM. Alveolar filling disorders. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 79.
Trapnell BC, Mccarthy C. Pulmonary alveolar proteinosis syndrome. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray & Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 98.