Treatment of Adult Primary Alveolar Proteinosis

Arch Bronconeumol. 2015 Jul;51(7):344-9. doi: 10.1016/j.arbres.2015.02.003. Epub 2015 Apr 17.
[Article in English, Spanish]


Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant-like lipoproteinaceous material in the distal air spaces and terminal bronchi, which may lead to impaired gas exchange. This accumulation of surfactant is due to decreased clearance by the alveolar macrophages. Its primary, most common form, is currently considered an autoimmune disease. Better knowledge of the causes of PAP have led to the emergence of alternatives to whole lung lavage, although this is still considered the treatment of choice. Most studies are case series, often with limited patient numbers, so the level of evidence is low. Since the severity of presentation and clinical course are variable, not all patients will require treatment. Due to the low level of evidence, some objective criteria based on expert opinion have been arbitrarily proposed in an attempt to define in which patients it is best to initiate treatment.

Keywords: Enfermedades raras; Factor estimulante de colonias de granulocitos-macrófagos; Granulocyte colony-stimulating factor-macrophage; Proteinosis alveolar pulmonar; Pulmonary alveolar proteinosis; Rare diseases; Tratamiento; Treatment.

Publication types

  • Review

MeSH terms

  • Adult
  • Allografts
  • Animals
  • Antibiotic Prophylaxis
  • Antibodies, Neutralizing / analysis
  • Antibodies, Neutralizing / immunology
  • Autoantibodies / analysis
  • Autoantibodies / immunology
  • Autoimmune Diseases / epidemiology
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / therapy*
  • Bronchoalveolar Lavage Fluid / immunology
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Disease Management
  • Disease Models, Animal
  • Granulocyte-Macrophage Colony-Stimulating Factor / deficiency
  • Granulocyte-Macrophage Colony-Stimulating Factor / physiology
  • Granulocyte-Macrophage Colony-Stimulating Factor / therapeutic use
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immunoglobulin G / analysis
  • Immunoglobulin G / immunology
  • Lung Transplantation
  • Macrophages / pathology
  • Mice
  • Mice, Knockout
  • Oxygen Inhalation Therapy
  • Plasmapheresis
  • Prospective Studies
  • Pulmonary Alveolar Proteinosis / epidemiology
  • Pulmonary Alveolar Proteinosis / genetics
  • Pulmonary Alveolar Proteinosis / immunology
  • Pulmonary Alveolar Proteinosis / therapy*
  • Pulmonary Gas Exchange
  • Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / deficiency
  • Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / physiology
  • Rituximab / therapeutic use
  • Therapeutic Irrigation


  • Antibodies, Neutralizing
  • Autoantibodies
  • Immunoglobulin G
  • Receptors, Granulocyte-Macrophage Colony-Stimulating Factor
  • Rituximab
  • Granulocyte-Macrophage Colony-Stimulating Factor