How is pulmonary alveolar proteinosis (PAP) treated?

The mainstay of PAP treatment for a very long time has been therapeutic whole-lung lavage (WLL). This procedure involves cleaning out one lung at a time with saline while the other lung is fed with oxygen. This procedure, performed under general anesthesia, usually takes from two to five hours.

Newer therapies being studied include:

  • Granulocyte-macrophage colony-stimulating factor (GM-CSF) given as shots under the skin or inhaled through a nebulizer
  • Rituximab, a monoclonal antibody, given as an intravenous (IV) infusion

If a person has secondary PAP, the underlying cause—such as a tumor—must be treated. If the cause is some type of exposure to a toxin or irritant, the exposure must first be stopped.

A person with PAP may be prescribed oxygen to help with breathing.

A bronchodilator (medication to improve air flow) might be prescribed if your type of PAP causes asthma symptoms.

In a few cases, a person with PAP is treated with a plasmapheresis or a lung transplant. Plasmapheresis removes blood plasma from the body, treats it, and then returns it to the body.

Rarely, someone with PAP will see the condition resolve on its own without treatment.

Last reviewed by a Cleveland Clinic medical professional on 06/06/2018.

References

  • Expert knowledge and experience of healthcare providers at Cleveland Clinic
  • Genetic and Rare Disease Information Center. Autoimmune Pulmonary Alveolar Proteinosis. Accessed 6/11/2018.
  • Borie R, Danel C, Debray MP, et al. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011;20(120):98-107.
  • National Organization for Rare Disorders. Pulmonary Alveolar Proteinosis. Accessed 6/11/2018.
  • Alshati MH. Alveolar proteinosis: a slow drowning in mud. Cleve Clin J Med. 2014; 81(8):468-470. doi:10.3949/ccjm.81a.13085.
  • Kumar A, Abdelmalak B, Inoue Y, Culver DA. Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach. The Lancet Respiratory Medicine. January 2018. doi:10.1016/s2213-2600(18)30043-2.

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