TY - CHAP
T1 - INFLAMMATION IN CYSTIC FIBROSIS
AU - Polineni, Deepika
AU - Chmiel, James F.
N1 - Publisher Copyright:
© 2024 selection and editorial matter, Andrew Bush, Margarida D. Amaral, Jane C. Davies, Nicholas J Simmonds, Jennifer L. Taylor-Cousar and Sarath Ranganathan; individual chapters, the contributors.
PY - 2023/1/1
Y1 - 2023/1/1
N2 - The topic of inflammation in cystic fibrosis (CF) is one of complex investigation due to multicellular contributors, as well as multifactorial associations in CF including relationships to intrinsic cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction, infection, and abnormal mucus properties. Although CFTR modulator therapies have markedly improved health for people with CF who are genetically able to benefit from these drugs and who have access to them, emerging evidence suggests that underlying CF inflammation persists. This chapter reviews the basic principles of CF inflammation including underlying relationships to CFTR dysfunction, cellular contributors and pathophysiology, associations with infection, and clinical manifestations. Technological advances in the study of CF inflammation are reviewed along with impacts of CFTR modulator treatment on CF inflammation. Established anti-inflammatory therapies are discussed along with the new challenges that must be addressed when considering future investigation of anti-inflammatory treatments.
AB - The topic of inflammation in cystic fibrosis (CF) is one of complex investigation due to multicellular contributors, as well as multifactorial associations in CF including relationships to intrinsic cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction, infection, and abnormal mucus properties. Although CFTR modulator therapies have markedly improved health for people with CF who are genetically able to benefit from these drugs and who have access to them, emerging evidence suggests that underlying CF inflammation persists. This chapter reviews the basic principles of CF inflammation including underlying relationships to CFTR dysfunction, cellular contributors and pathophysiology, associations with infection, and clinical manifestations. Technological advances in the study of CF inflammation are reviewed along with impacts of CFTR modulator treatment on CF inflammation. Established anti-inflammatory therapies are discussed along with the new challenges that must be addressed when considering future investigation of anti-inflammatory treatments.
UR - http://www.scopus.com/inward/record.url?scp=85190628457&partnerID=8YFLogxK
U2 - 10.1201/9781003262763-8
DO - 10.1201/9781003262763-8
M3 - Chapter
AN - SCOPUS:85190628457
SN - 9781032202204
SP - 74
EP - 86
BT - Hodson and Geddes’ Cystic Fibrosis, Fifth Edition
PB - CRC Press
ER -