TY - JOUR
T1 - Respiratory tract exacerbations revisited
T2 - Ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatment
AU - Tiddens, Harm A.W.M.
AU - Stick, Stephen M.
AU - Wild, Jim M.
AU - Ciet, Pierluigi
AU - Parker, Geoffrey J.M.
AU - Koch, Armin
AU - Vogel-Claussen, Jens
N1 - Publisher Copyright:
© 2015 Wiley Periodicals, Inc.
PY - 2015/10/1
Y1 - 2015/10/1
N2 - For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in the progressive loss of functional lung tissue. There is a poor understanding of the pathobiology of RTE and whether specific treatment of RTE reduces lung damage in the long term. In addition, there are limited tools available to measure the various components of CF lung disease and responses to therapy. Therefore, in order to better understand the impact of RTE on CF lung disease we need to develop sensitive measures to characterize RTE and responses to treatment; and improve our understanding of structure-function changes during treatment of RTE. In this paper we review our current knowledge of the impact of RTE on the progression of lung disease and identify strategies to improve our understanding of the pathobiology of RTE. By improving our knowledge regarding RTE in CF we will be better positioned to develop approaches to treatment that are individualized and that can prevent permanent structural damage. We suggest the development of a ventilation, perfusion, inflammation and structure (VIPS)-MRI suite that supplies the clinician with data on ventilation, inflammation, perfusion, and structure in one MRI session. VIPS-MRI could be an important step to better understand the factors that contribute to and limit treatment efficacy of RTE.
AB - For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in the progressive loss of functional lung tissue. There is a poor understanding of the pathobiology of RTE and whether specific treatment of RTE reduces lung damage in the long term. In addition, there are limited tools available to measure the various components of CF lung disease and responses to therapy. Therefore, in order to better understand the impact of RTE on CF lung disease we need to develop sensitive measures to characterize RTE and responses to treatment; and improve our understanding of structure-function changes during treatment of RTE. In this paper we review our current knowledge of the impact of RTE on the progression of lung disease and identify strategies to improve our understanding of the pathobiology of RTE. By improving our knowledge regarding RTE in CF we will be better positioned to develop approaches to treatment that are individualized and that can prevent permanent structural damage. We suggest the development of a ventilation, perfusion, inflammation and structure (VIPS)-MRI suite that supplies the clinician with data on ventilation, inflammation, perfusion, and structure in one MRI session. VIPS-MRI could be an important step to better understand the factors that contribute to and limit treatment efficacy of RTE.
KW - lung disease
KW - magnetic resonance imaging
KW - pathobiology
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U2 - 10.1002/ppul.23266
DO - 10.1002/ppul.23266
M3 - Review article
C2 - 26335955
AN - SCOPUS:84941087623
SN - 8755-6863
VL - 50
SP - S57-S65
JO - Pediatric Pulmonology
JF - Pediatric Pulmonology
ER -