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Related Concept Videos

Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

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Type I Respiratory Failure, or hypoxemic respiratory failure, occurs when the partial pressure of oxygen (PaO2) in arterial blood falls below 60 mmHg while breathing room air without a corresponding increase in arterial carbon dioxide levels (PaCO2). This condition highlights a significant impairment in the lungs' capacity to oxygenate the blood.
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Related Experiment Video

Updated: Dec 22, 2025

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
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Pulmonary alveolar proteinosis.

Stéphane Jouneau1,2, Cédric Ménard3, Mathieu Lederlin4,5

  • 1Department of Respiratory Medicine, Competence Centre for Rare Pulmonary Diseases, CHU Rennes, Univ Rennes, Rennes, France.

Respirology (Carlton, Vic.)
|May 5, 2020
PubMed
Summary

Pulmonary alveolar proteinosis (PAP) is a rare lung disease where surfactant accumulates in the alveoli. Treatment involves whole lung lavage (WLL) and inhaled granulocyte-macrophage colony-stimulating factor (GM-CSF).

Keywords:
granulocyte-macrophage colony-stimulating factorinfectionpulmonary alveolar proteinosisrituximabwhole lung lavage

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Area of Science:

  • Pulmonary Medicine
  • Rare Diseases
  • Immunology

Background:

  • Pulmonary alveolar proteinosis (PAP) is an ultra-rare lung disease characterized by surfactant component accumulation in alveoli, impairing gas exchange.
  • Primary PAP, often autoimmune, involves anti-GM-CSF antibodies and constitutes over 90% of cases. Secondary and genetic forms also exist.
  • Diagnosis involves characteristic chest CT findings (crazy-paving pattern) and bronchoalveolar lavage (BAL) analysis.

Purpose of the Study:

  • To summarize the understanding of pulmonary alveolar proteinosis (PAP), including its types, diagnosis, treatment, and complications.
  • To highlight current therapeutic strategies and their efficacy.
  • To provide an overview of the disease's clinical course and prognosis.

Main Methods:

  • Literature review and synthesis of existing data on PAP.
  • Analysis of diagnostic criteria, including imaging and laboratory findings.
  • Evaluation of treatment outcomes for various therapeutic interventions.

Main Results:

  • Autoimmune PAP, identified by anti-GM-CSF antibodies, is the most common form.
  • Whole lung lavage (WLL) remains the first-line treatment, with inhaled GM-CSF as a second-line option, showing better efficacy than subcutaneous administration.
  • Complications primarily involve infections, and the 5-year survival rate is 95%.

Conclusions:

  • PAP diagnosis relies on characteristic imaging and BAL findings, with surgical lung biopsy rarely needed.
  • Inhaled GM-CSF offers a promising second-line treatment, superior to other therapies like rituximab or plasmapheresis.
  • Despite unpredictable clinical courses, PAP has a favorable long-term survival rate, though infection risk necessitates careful management.