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Advanced Pulmonary Alveolar Proteinosis (PAP)

Whole-lung lavage and inhaled GM-CSF therapy for refractory or severe pulmonary alveolar proteinosis.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

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 Advanced Pulmonary Alveolar Proteinosis (PAP)?

Pulmonary alveolar proteinosis (PAP) is a rare disease caused by accumulation of surfactant-derived lipoproteinaceous material in alveoli, classified as autoimmune (anti-GM-CSF antibodies), hereditary (CSF2RA/CSF2RB mutations) or secondary to hematologic disease, dust exposure or immunodeficiency.

Patients present with progressive dyspnea, dry cough, hypoxemia and characteristic crazy-paving pattern on HRCT; bronchoalveolar lavage shows milky fluid with PAS-positive proteinaceous material.

Advanced therapy is reserved for symptomatic, hypoxemic or progressive disease and includes general anesthesia whole-lung lavage with up to 20-40 liters of saline per lung, inhaled or subcutaneous GM-CSF (sargramostim, molgramostim), plasmapheresis and rituximab in selected refractory autoimmune cases.

Symptoms

Progressive exertional dyspnea
Dry cough
Hypoxemia and cyanosis
Fatigue and weight loss
Crackles on auscultation
Recurrent secondary infections
Clubbing in chronic cases

Risk Factors

Anti-GM-CSF autoantibodies
Smoking
Silica or dust exposure
Hematologic malignancies
Immunodeficiency syndromes
Hereditary CSF2RA/CSF2RB mutations
Heavy occupational dust exposure

When to See a Doctor?

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

  • Persistent dyspnea and hypoxemia
  • Crazy-paving pattern on chest CT
  • Recurrent respiratory infections
  • Need for whole-lung lavage in tertiary center
  • Suspected refractory autoimmune PAP
  • Severe disease with rapid progression
  • Pre-treatment evaluation for inhaled GM-CSF

Treatment Methods

01
Confirmation of subtype (anti-GM-CSF antibodies, genetic testing)
02
Whole-lung lavage under general anesthesia in tertiary center
03
Inhaled or subcutaneous GM-CSF (sargramostim, molgramostim)
04
Plasmapheresis or rituximab in refractory autoimmune disease
05
Treatment of underlying hematologic or immunologic disease
06
Smoking cessation and infection control
07
Lung transplantation in end-stage disease

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.