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Pulmonary Alveolar Proteinosis

Shannon L McCown, MD Reviewed 05/2023
 


BASICS

DESCRIPTION

  • Pulmonary alveolar proteinosis (PAP) or pulmonary alveolar phospholipoproteinosis, first described in 1958 by Rosen et al, is characterized by overproduction of abnormal, nonfunction...

DIAGNOSIS

HISTORY

  • Diagnosis often delayed due to nonspecific presentation, therefore, high clinical suspicion with thorough occupational and exposure history should be obtained (2)

  • Clinical manifestati...

TREATMENT

  • Management is based on disease progression, coexisting infections, and degree of physiologic impairment (5,6).

  • In general, patient is classified as asymptomatic or symptomatic. The latter mi...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Serial serum LDH level, BAL IgG anti–GM-CSF concentration, and chest X-ray can correlate with disease activity. KL-6 in serum could be used for disease progressio...

REFERENCES

1
Salvaterra E, Campo I. Pulmonary alveolar proteinosis: from classification to therapy. Breathe (Sheff). 2020;16(2):200018. doi: 10.1183/20734735.0018-2020.
2
Kumar A, Abdelmalak B, Inoue Y, ...

ADDITIONAL READING

CODES

ICD10

J84.01 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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