Lege Artis Medicinae

[AUTOIMMUNE PATHOGENESIS IN THE BACKGROUND OF PULMONARY ALVEOLAR PROTEINOSIS]

LOSONCZY György

JANUARY 21, 2006

Lege Artis Medicinae - 2006;16(01)

[Pulmonary alveolar proteinosis (PAP) is a rare form of interstitial lung disease. The etiology and pathogenesis of PAP has remained uncertain. It is characterized by the accumulation of surfactant in alveolar space, which leads to impaired oxygen diffusion and lung restriction. Patients develop chronic respiratory failure. Traditional treatment of the disease has been whole-lung lavage. In recent years the role of alveolar macrophages in the removal of surfactant has been discovered with granulocyte-macrophage colony-stimulating factor (GM-CSF) triggering this function of macrophages. In the most frequent “idiopathic” subtype of this disease autoantibodies against GM-CSF have been shown to be present in blood. These autoantibodies block GM-CSF-induced surfactant removal by the alveolar macrophages. Clinical studies indicated that longterm, high dose therapy with GM-CSF can be effective in the cleaning the alveolar space and treating respiratory failure. This new treatment modality makes high-risk whole-lung lavage avoidable.]

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