Cytokines and Inflammatory Mediators in Cystic Fibrosis

Abstract

Cystic fibrosis (CF), a multisystem disorder of children and adults is the most common life-threatening genetic trait in the Caucasian population. It is the major cause of severe chronic lung disease in children. Alteration of immunologic functions has been demonstrated and may play a role in the progressive pulmonary deterioration resulting in inflammation, fibrosis, and loss of function. Pulmonary inflammatory disease involves many interacting cells that release cytokines that activate several target cells of the respiratory tract. Various cytokines such as interleukin-6 (IL-6) and interleukin-8 (IL-8), platelet activating factor (PAF), and leukotriene B4 (LTB4) may be important in patients with CF who have acute and chronic purulent sputum production and exacerbation of respiratory symptoms requiring intravenous antibiotic therapy. Investigators are currently investigating cytokines and other inflammatory mediators in CF as well as in asthma. Questions remain to be answered as to the role that cytokines and inflammatory mediators play in the extent of inflammation and pulmonary damage in the cystic fibrosis population.