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Pulmonary alveolar proteinosis - Treatment

Alternative Names

Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis

Treatment:

Treatment involves washing out the protein substance from the lung (whole-lung lavage) from time to time. Certain patients with this disease may need to have a lung transplant.

Research has shown some benefit to an experimental treatment that uses a blood-stimulating medication called granulocyte-macrophage colony stimulating factor (GM-CSF), which is known to be lacking in some patients with alveolar proteinosis.

Expectations (prognosis):

Some people with this condition go into remission. Others have respiratory failure that gets worse, and they may need a lung transplant. Up to 25% of people with this condition die within 5 years of being diagnosed.

Calling your health care provider:

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

  • Reviewed last on: 6/10/2011
  • David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Denis Hadjiliadis, MD, Associate Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Levine SM. Alveolar filling disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 91.

Trapnell BC, Nakata K, Kavuru MS. Pulmonary alveolar proteinosis syndrome. In: Mason RJ, Broaddus VC, Martin TR, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 63.

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