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Pulmonary Alveolar Proteinosis (PAP)

A rare lung disease characterized by accumulation of surfactant-like protein-lipid material in the alveoli.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Pulmonary Alveolar Proteinosis (PAP)?

Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of surfactant-like proteinaceous material in the alveoli. There are autoimmune (GM-CSF antibodies), secondary, and congenital forms. The autoimmune form is the most common in adults.

Alveolar macrophages cannot clear surfactant due to GM-CSF deficiency, and the alveoli become filled. This impairs gas exchange and leads to hypoxia. Patients typically present with prolonged cough and dyspnea.

Diagnosis is made by 'crazy paving' appearance on CT, milky white material in BAL, and PAS-positive proteinaceous substance. Treatment includes whole lung lavage and inhaled GM-CSF therapy.

Symptoms

Progressive dyspnea
Dry cough
Fatigue
Weight loss
Cyanosis
Rarely fever
Secondary infections

Risk Factors

Autoimmune GM-CSF antibodies
Silica exposure
Hematological malignancies
Immunodeficiency states
Genetic mutations (in children)
Bone marrow transplantation
Severe infections

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Chronic progressive dyspnea
  • Dry cough and weight loss
  • Crazy paving pattern on CT
  • Development of hypoxia

Treatment Methods

01
Whole lung lavage
02
Inhaled GM-CSF therapy
03
Treatment of underlying cause in secondary forms
04
Oxygen support
05
Vaccination and infection control
06
Rituximab and plasmapheresis (in resistant cases)

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Göğüs Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.