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Pulmonary Alveolar Proteinosis

Reviewed 06/2019
 


BASICS

DESCRIPTION

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

DIAGNOSIS

HISTORY

  • Diagnosis often delayed due to nonspecific presentation, therefore, high clinical suspicion and occupational and exposure history should be obtained.

  • Most common reported symptoms: dys...

TREATMENT

  • Management is based on disease progression, coexisting infections, and degree of physiologic impairment (signs of poor gas exchange).

  • In general, patient is classified as asymptomatic or symp...

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
Huaringa A, Francis W. Pulmonary alveolar proteinosis: a case report and world literature review. Respirol Case Rep.  2016;4(6):e00201. [View Abstract on OvidMedline]
2
Ben-Dov I, Segel...

ADDITIONAL READING

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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