Pulmonary Alveolar Proteinosis
Also written as: PAP — Pulmonary Alveolar Proteinosis
A rare lung disorder characterised by the accumulation of surfactant-derived lipoproteinaceous material within alveoli, impairing gas exchange.
Full Definition
Pulmonary alveolar proteinosis (PAP) in children may be congenital, autoimmune, or secondary to infection or immunodeficiency. The alveoli fill with a periodic acid–Schiff (PAS)-positive proteinaceous material that results from impaired surfactant clearance by alveolar macrophages. Congenital PAP is associated with mutations in surfactant protein genes (SP-B, SP-C) or the GM-CSF receptor. Editors should note that this condition is distinct from surfactant deficiency disorders; in PAP, surfactant is overproduced or under-cleared rather than absent. The standard treatment in older children is whole-lung lavage.
Usage
Usage note: Expand acronym PAP on first use; distinguish clearly from pulmonary arterial pressure (also PAP) in manuscripts covering cardiopulmonary topics.
In Context
- "The radiologist's report described bilateral ground-glass opacities with 'crazy-paving' pattern, prompting further workup that confirmed pulmonary alveolar proteinosis." — Radiology report
- "Authors must not conflate pulmonary alveolar proteinosis with surfactant deficiency, as the underlying pathophysiology and treatment differ substantially." — Manuscript review note