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Pulmonary Alveolar Proteinosis

 


BASICS

DESCRIPTION

  • Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by accumulation of lipoproteinaceous (surfactant-like) material in the alveolar spaces, leading to impaired gas e...

DIAGNOSIS

HISTORY

  • Diagnosis often delayed due to nonspecific presentation

  • Occupational and exposure history to exclude secondary PAP

  • Symptoms

    • Progressive dyspnea with insidious onset (most common presenta...

TREATMENT

GENERAL MEASURES

  • Supportive measures such as supplemental oxygen, bronchodilators, and antibiotics for infection, smoking cessation, and treatment of concurrent diseases that impair respirato...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Respiratory infections are a common complication of PAP, especially with atypical organisms such as Nocardia spp. or Pneumocystis jirovecii. Any...

ADDITIONAL READING

  • Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J.  2004;23(4):526–531.

  • Chung MJ, Lee KS, ...

CODES

ICD10

J84.01 Alveolar proteinosis 

ICD9

516.0 Pulmonary alveolar proteinosis 

SNOMED

10501004 pulmonary alveolar proteinosis (disorder) 

CLINICAL PEARLS

  • PAP is a rare disease characterized by accumulation of PAS-positive surfactant-like material in the alveolar spaces leading to impaired gas exchange.

  • Nonspecific respiratory symptoms an...

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