Abstract
Cystic fibrosis is characterized by excessive pulmonary inflammation, which presents early in life and becomes self-sustaining, eventually leading to the destruction of the lung. Treating inflammation is one of the most pressing needs in CF therapy and has been shown to slow lung function deterioration. However, it remains unclear whether excessive inflammation is a direct result of CFTR dysfunction, and thus innate, or develops in response to early stimulation of inflammatory pathways. Here, we will discuss clinically relevant studies and the methods employed by them. We will focus on investigations in cell and animal models as well as patients. Our discussion will describe the character of pulmonary inflammation in CF and present potential therapeutic approaches that can ameliorate excessive responses and improve disease prognosis.
MeSH terms
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Animals
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Anti-Inflammatory Agents / therapeutic use
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Blotting, Western
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Cell Culture Techniques
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Cystic Fibrosis / complications
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Cystic Fibrosis / drug therapy
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Cystic Fibrosis / immunology
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Cystic Fibrosis / metabolism
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Cystic Fibrosis / physiopathology*
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Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
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Cytokines / immunology
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Cytokines / metabolism
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Disease Models, Animal
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Electrophoresis, Polyacrylamide Gel
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Humans
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Immunohistochemistry
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Inflammation / complications
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Inflammation / drug therapy
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Inflammation / immunology
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Inflammation / metabolism
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Inflammation / physiopathology*
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Lipopolysaccharides / immunology
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Lung / metabolism
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Lung / physiopathology
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Mice
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Oligonucleotide Array Sequence Analysis
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Pseudomonas aeruginosa / chemistry
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Transcription Factors / genetics
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Transcription Factors / metabolism
Substances
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Anti-Inflammatory Agents
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Cytokines
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Lipopolysaccharides
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Transcription Factors
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Cystic Fibrosis Transmembrane Conductance Regulator