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Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in the alveolar space. According to the underlying pathogenetic mechanisms, three different forms have been identified, namely primary, secondary and congenital. Primary PAP is caused by disruption of granulocyte−macrophage colony-stimulating factor (GM-CSF) signalling due to the presence of neutralising autoantibodies (autoimmune PAP) or GM-CSF receptor genetic defects (hereditary PAP), which results in dysfunctional alveolar macrophages with reduced phagocytic clearance of particles, cholesterol and surfactant. The serum level of GM-CSF autoantibody is the only disease-specific biomarker of autoimmune PAP, although it does not correlate with disease severity. In PAP patients with normal serum GM-CSF autoantibody levels, elevated serum GM-CSF levels is highly suspicious for hereditary PAP. Several biomarkers have been correlated with disease severity, although they are not specific for PAP. These include lactate dehydrogenase, cytokeratin 19 fragment 21.1, carcinoembryonic antigen, neuron-specific enolase, surfactant proteins, Krebs von Lungen 6, chitinase-3-like protein 1 and monocyte chemotactic proteins. Finally, increased awareness of the disease mechanisms has led to the development of pathogenesis-based treatments, such as GM-CSF augmentation and cholesterol-targeting therapies.
Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in the alveolar space. According to the underlying pathogenetic mechanisms, three different forms have been identified, namely primary, secondary and congenital. Primary PAP is caused by disruption of granulocyte−macrophage colony-stimulating factor (GM-CSF) signalling due to the presence of neutralising autoantibodies (autoimmune PAP) or GM-CSF receptor genetic defects (hereditary PAP), which results in dysfunctional alveolar macrophages with reduced phagocytic clearance of particles, cholesterol and surfactant. The serum level of GM-CSF autoantibody is the only disease-specific biomarker of autoimmune PAP, although it does not correlate with disease severity. In PAP patients with normal serum GM-CSF autoantibody levels, elevated serum GM-CSF levels is highly suspicious for hereditary PAP. Several biomarkers have been correlated with disease severity, although they are not specific for PAP. These include lactate dehydrogenase, cytokeratin 19 fragment 21.1, carcinoembryonic antigen, neuron-specific enolase, surfactant proteins, Krebs von Lungen 6, chitinase-3-like protein 1 and monocyte chemotactic proteins. Finally, increased awareness of the disease mechanisms has led to the development of pathogenesis-based treatments, such as GM-CSF augmentation and cholesterol-targeting therapies.
Biologická léčiva navzdory rozmachu využití v jiných oblastech pneumologie nebývají léky první volby u nemocných s intersticiálními plicními procesy. Důvodem je vzácnější výskyt těchto nemocí a jejich neúplně objasněná etiopatogeneze. Nejčastěji jsou biologika používána u nemocných s intersticiálním plicním postižením při systémových chorobách pojiva, naopak u nemocných s idiopatickými intersticiálními pneumoniemi svoje místo v rutinní léčbě nemají. U pacientů se sarkoidózou tvoří biologická léčba 3. linii terapie. Před zahájením biologické léčby je třeba vzít v potaz i její rizika a pacienta adekvátně vyšetřit, zejména ve vztahu k riziku reaktivace některých infekcí.Klíčová slova: biologická léčba, intersticiální plicní procesy, idiopatická plicní fibróza, sarkoidóza, systémové choroby pojiva. Biological therapy of interstitial lung diseasesBiological therapies, despite their widespread use in other areas of pneumology, are not typically the first-line treatment for patients with interstitial lung diseases. This is due to the relatively rare occurrence of these diseases and their incompletely understood etiopathogenesis. Biologics are most commonly used in patients with interstitial lung involvement in systemic connective tissue diseases; conversely, they do not have a routine place in the treatment of patients with idiopathic interstitial pneumonias. In patients with sarcoidosis, biological therapy constitutes third-line treatment. Before initiating biological treatment, it is necessary to consider its risks and thoroughly evaluate the patient, particularly regarding the risk of reactivation of certain infections.
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