Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by excessive accumulation of surfactant components in alveolar macrophages, alveoli, and peripheral airways. The accumulation of surfactant is associated with only a minimal inflammatory response but can lead to the development of pulmonary fibrosis. Three clinical forms of PAP are distinguished - primary, secondary and congenital. In recent years, significant findings have helped to clarify the ethiology and pathogenesis of the disease. Apart from impaired surfactant protein function, a key role in the development of PAP is played by signal pathway of granulocyte and macrophage colonies stimulating growth factor (GM-CSF) which is necessary for the functioning of alveolar macrophages and for surfactant homeostasis. Surfactant is partially degraded by alveolar macrophages that are stimulated by GM-CSF. The role of GM-CSF has been shown especially in primary PAP, which is currently considered an autoimmune disease involving the development of GM-CSF neutralising autoantibodies. Clinically, the disease may be silent or manifest with dyspnoeic symptoms triggered by exertion and cough. However, there is a 10 to 15% rate of patients who develop respiratory failure. Total pulmonary lavage is regarded as the standard method of treatment. In addition, recombinant human GM-CSF has been studied as a prospective therapy for the treatment of PAP.

1st Department of Pneumology and Phthisiology National Institute for Tuberculosis Lung Diseases and Thoracic Surgery in Vyšné Hágy Vysoké Tatry Slovakia

2nd Department of Pneumology and Phthisiology National Institute for Tuberculosis Lung Diseases and Thoracic Surgery in Vyšné Hágy Vysoké Tatry Slovakia

Cancer Research Institute Biomedical Research Centre of the Slovak Academy of Sciences Bratislava Slovakia

Centre for Thoracic Surgery National Institute for Tuberculosis Lung Diseases and Thoracic Surgery in Vyšné Hágy Vysoké Tatry Slovakia

Department of Anatomy of the Faculty of Medicine of Pavol Jozef Šafárik University in Košice Slovakia

Department of Pathology National Institute for Tuberculosis Lung Diseases and Thoracic Surgery in Vyšné Hágy Vysoké Tatry Slovakia

Institute of Clinical and Molecular Pathology and Medical Genetics Faculty of Medicine University of Ostrava Ostrava Czech Republic

Institute of Histology and Embryology Faculty of Medicine University of Ostrava Ostrava Czech Republic

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