DISEASES
description
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by an abnormal accumulation of surfactant-derived lipoprotein compounds within the lung alveoli (air sacs). The accumulated substances interfere with normal gas exchange and expansion of the lungs, ultimately leading to difficulty breathing and a predisposition to lung infections. The signs and symptoms of PAP include shortness of breath, a cough, low grade fever, and weight loss. PAP is primarily caused by reduced GM-CSF stimulation. GM-CSF is a cytokine protein that stimulates alveolar macrophages to function properly and maintain a normal surfactant level in alveoli by removing excess surfactant.
PAP can arise as an autoimmune disease (autoantibodies against GM-CSF) or as a hereditary disease (genetic mutation of GM-CSF receptors). Mitogen’s Alveolar Proteinosis Test (Anti‐GMCSF) aids in the diagnosis of autoimmune PAP.
Symptoms
- Shortness of breath, also called dyspnea
- Chest pain or tightness
- Fever
- Weight loss
- Cough (sometimes, but not always)
- Low levels of oxygen in the blood
- Nail clubbing (abnormal growth of toenails or fingernails)