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Pulmonary alveolar proteinosis
Other Resources UpToDate PubMed

Pulmonary alveolar proteinosis

Contributors: Casey Silver MD, Mary Anne Morgan MD, Michael W. Winter MD
Other Resources UpToDate PubMed

Synopsis

Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material. This material consists of surfactant phospholipids and apoproteins and stains positive with periodic acid-Schiff (PAS) stain. Alveolar architecture is typically preserved, and inflammatory cell infiltration is minimal.

There are 3 categories of the condition:
  1. Autoimmune and hereditary PAP – Causes disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF).
  2. Congenital PAP – Genetic disorder of surfactant production; presents in neonates.
  3. Secondary PAP – Associated with mineral dust exposure, hematologic malignancies, and allogenic hematopoietic cell transplantation.
Typical age of presentation in adults with PAP is between 40 and 50 years. Smoking is a significant risk factor. Some patients will have other autoimmune diseases.

Signs and symptoms include dyspnea, particularly on exertion, cough, and low-grade fever. The classic radiographic pattern is "crazy paving" on high-resolution CT, but other patterns may be observed.

Neonates with congenital PAP present in the first 1-2 years of life with symptoms ranging from severe respiratory failure in the neonatal period to insidious chronic interstitial lung disease in later months.

Codes

ICD10CM:
J84.01 – Alveolar proteinosis

SNOMEDCT:
10501004 – Pulmonary alveolar proteinosis

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Therapy

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Drug Reaction Data

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References

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Last Reviewed:07/11/2019
Last Updated:07/16/2019
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Pulmonary alveolar proteinosis
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A medical illustration showing key findings of Pulmonary alveolar proteinosis : Exertional dyspnea, Malaise, Pleuritic chest pain, Low grade fever, Dry cough
Copyright © 2024 VisualDx®. All rights reserved.