Pulmonary alveolar proteinosis
Journal Title: Postępy Nauk Medycznych - Year 2011, Vol 24, Issue 4
Abstract
Pulmonary alveolar proteinosis (PAP) is an “orphan lung disorder” characterized by abundant accumulation of surfactant-derived phospholipids and protein components in the alveoli and distal airways. PAP was first described in 1958, and for many years the pathogenesis of the disease was unknown. Three clinically and etiologically distinct forms of PAP have been recognized (congenital, secondary and idiopathic), but more then 90% of the cases are idiopathic. Idiopathic PAP is considered to be caused by an autoantibody to the granulocyte-macrophage colony stimulating factor (GM-CSF), which neutralizes GM-CSF and therefore impairs the differentiation of alveolar macrophages. The condition has a variable clinical course from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infection. The diagnosis of PAP may be strongly supported by high-resolution computed tomography (HRCT) of the chest, which reveals diffuse ground-glass opacification superimposed on septal thickening (commonly referred to as “crazy paving”) with geographical distribution.No specific therapy exists for PAP and whole lung lavage remains the standard of care. Exogenous therapy with GM-CSF may improve the lung disease in some patients with PAP but this therapy is still experimental.
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Małgorzata Sobiecka
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