Severe Pulmonary Alveolar Proteinosis in a Young Adult

Mild dyspnea presenting as ‘crazy-paving’ on chest computed tomography

“…It is a disorder characterized by surfactant, lipid and protein accumulation in the alveolar spaces [3]. The etiology of PAP is varied and thought to be due to either autoimmune, secondary/exposure, and/or congenital causes [4,5]. Autoimmune PAP compromises 90% of the cases and it is a result of anti-granulocytemacrophage colony-stimulating factor (GM-CSF) antibodies that lead to a deficiency of GM-CSF [6].…”

COVID-19 and severe pulmonary alveolar proteinosis (PAP): A case report

“…Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of surfactant proteins within the alveoli due to either increased surfactant production or decreased clearance. When PAP is clinically suspected, a bronchoalveolar lavage (BAL) in addition to computed tomography (CT) scan typical presentation is often sufficient for the diagnosis of PAP [ [1] , [2] , [3] , [4] ]. Total or whole lung lavage (WLL) is considered the mainstay treatment in symptomatic patients.…”

Pulmonary Alveolar Proteinosis and Pregnancy: A Review of the Literature and Case Presentation

“…It is mediated by immunoglobulin G (IgG) anti-granulocyte macrophage colony stimulating factor (anti-GM-CSF) antibodies, which cause a decrease in alveolar macrophages’ function [ 2 ]. Increased levels of anti-GM-CSF are found in the bronchoalveolar lavage fluid in PAP patients [ 1 ], and the diagnosis is based on the examination of this fluid or transbronchial biopsies [ 2 ]. Surfactant clearance under normal conditions and abnormal clearance in PAP is illustrated in Figure 1 .…”