Lymphocyte dysregulation plays an important role in pathogenesis of cystic fibrosis

Abstract

Cystic fibrosis (CF) is the most common genetic disorder in the Caucasian population. CF is usually associated with lung disease and patients suffer from persistent infections with strong pulmonary inflammation. It has been shown that fluid and electrolyte transport across epithelial membranes is affected in CF. However, the single gene that is affected is widely expressed in many tissues, including immune cells such as T-lymphocytes. The purpose of this thesis is to analyse available literature on the influence of the genetic defect in CF on the adaptive immune system. The idea that the immune system is involved is not new, but recently new insights supporting this hypothesis have been published. These indicate that the CF immune system is already in a proinflammatory state and prone to a TH2 response before contact with microorganisms.