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Pulmonary Alveolar Proteinosis

Abstract

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NORD is very grateful to Christopher Towe, MD and Bruce Trapnell, MS, MD, Cincinnati Children's Hospital Medical Center, for assistance in the preparation of this report.

Synonyms of Pulmonary Alveolar Proteinosis

  • Alveolar Lipoproteinosis Phospholipidosis
  • PAP

Disorder Subdivisions

  • Congenital PAP (multiple diseases, usually genetic)
  • Primary PAP (autoimmune PAP, hereditary PAP)
  • Secondary PAP (multiple diseases)

General Discussion

The lung is composed of millions of tiny air sacs (alveoli) with very thin walls that allow oxygen in the air we breathe to pass through into the blood. Surfactant is a natural substance consisting of fat (mostly phospholipids) and a small amount of protein made in alveoli. Normally, a thin layer of surfactant present on the surface of alveoli helps them stay open. This allows air to come in and out as we breathe. Once used, surfactant is removed (cleared) from alveoli by cells called alveolar macrophages. This helps prevent surfactant from building up too much. Alveolar macrophages require a protein called granulocyte/macrophage-colony stimulating factor (GM-CSF) to maintain a normal surfactant level in alveoli (a process called surfactant homeostasis).

Pulmonary alveolar proteinosis (PAP) is a syndrome, a set of symptoms and signs and not just a single disease, in which surfactant slowly builds up in alveoli. This blocks air from entering alveoli and oxygen from passing through into the blood, which results in a feeling of breathlessness (dyspnea). Research has greatly improved our understanding of the diseases that cause PAP and how to identify (diagnose) and treat them. Diseases that cause PAP can occur in men, women, and children of all ages, ethnic backgrounds, and geographic locations. Disease severity varies from mild to severe and depends on which disease is present. Thus, it is important to know which disease is causing PAP in order to determine the best therapy and expected treatment response. Diseases that cause PAP can be grouped into three categories: primary PAP, secondary PAP, and congenital PAP (more accurately called disorders of surfactant production).

Pulmonary Alveolar Proteinosis Resources

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

Organizations:

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