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Alveolar proteinosis

Alveolar proteinosis is a rare pathology (1:4 million) that occurs as a result of accumulation of protein-lipid complexes in lung cells (alveoli) due to impaired surfactant processing, deterioration of gas exchange and the development of progressive respiratory failure. Usually middle-aged people get sick. Men get sick more often than women (1:3).

3 forms of the disease:Genetic form

  • The secondary form (pseudoproteinosis) can develop as a result of infections, diseases of the hematopoietic system, exposure to toxic fumes, inorganic dust.
  • Autoimmune idiopathic form (the most common).

You can suspect the disease if the following symptoms occur:Gradual onset, often accidental detection during X-ray examination

  • Increasing shortness of breath during physical exertion
  • Unproductive cough
  • Discomfort in the chest
  • Subfebrile temperature
  • Rarely hemoptysis
  • 40% of patients are initially diagnosed with bilateral pneumonia, tuberculosis.

If one or more of these signs are detected, it is recommended to consult a doctor. The specialist will assess the situation and appoint a follow-up examination.

The diagnosis of alveolar proteinosis is established by the totality of the clinical picture and the results of laboratory and instrumental studies.

To diagnose alveolar proteinosis,:laboratory tests: general and biochemical analysis of blood, examination of arterial blood gases, determination of the titer of antibodies to GM-GFR (granulocyte-macrophage colony stimulating factor);

  • instrumental studies: multispiral computed tomography, examination of the function of external respiration (bodyplethysmography, diffusion test), bronchoscopy with bronchoalveolar lavage (BAL).

The main treatment of alveolar proteinosis is to perform therapeutic bronchoalveolar lavage (performed under general anesthesia).

After the BAL procedure, clinical, radiological and functional improvements are noted. Most patients remain in long-term remission.

GBUZ Moscow Clinical Scientific Center named after Loginov MHD