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Pulmonary alveolar proteinosis - Overview

Alternative Names

Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis

Definition of Pulmonary alveolar proteinosis:

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

Causes, incidence, and risk factors:

In some cases, the cause of pulmonary alveolar proteinosis 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.

This rare disorder generally affects people ages 30 - 50 and is seen in men more often than in women. A form of the disorder is present at birth (congenital).

  • 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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