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[Pulmonary alveolar proteinosis].

Lucila A Pensa1, Martín R Solernó1, Silvia Rausch2

  • 1Servicio de Clínica Médica, Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires, Argentina.

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|April 27, 2021
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Summary

Pulmonary alveolar proteinosis (PAP), a rare lung disease, can cause respiratory failure. Early diagnosis and whole-lung lavage led to clinical improvement in a 69-year-old woman.

Keywords:
macrophagepulmonary alveolar proteinosispulmonary lavagesurfactant

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Area of Science:

  • Pulmonology
  • Rare Diseases
  • Critical Care Medicine

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease characterized by abnormal surfactant accumulation in the alveoli.
  • It presents with symptoms like progressive dyspnea and non-productive cough, potentially leading to respiratory failure.

Observation:

  • A 69-year-old woman presented with a three-month history of worsening dyspnea and cough.
  • Diagnostic workup revealed Type I respiratory failure, with chest tomography showing characteristic findings of septal thickening, ground-glass opacities, and bilateral consolidation.

Findings:

  • Histological examination confirmed the diagnosis of Pulmonary Alveolar Proteinosis.
  • The patient underwent whole-lung lavage, a therapeutic procedure for PAP, resulting in significant clinical improvement.

Implications:

  • This case underscores the importance of considering rare conditions like PAP in the differential diagnosis of respiratory failure and pulmonary infiltrates.
  • Maintaining a broad differential diagnosis is crucial, especially during the COVID-19 pandemic when overlapping symptoms can obscure less common diseases.