Respiratory tract exacerbations revisited: ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatment.
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.
Department of Pediatric Pulmonology and Allergology, Erasmus Medical Centre-Sophia Children's Hospital, Rotterdam, the Netherlands.|Department of Radiology, Erasmus Medical Centre, Rotterdam, the Netherlands.
Harm A W M
HA
Tiddens
Telethon Institute for Child Health Research, The University of Western Australia, Perth, Australia.|School of Paediatrics and Child Health Research, The University of Western Australia, Perth, Australia.
Stephen M
SM
Stick
Department of Academic Radiology, University of Sheffield, UK.
Jim M
JM
Wild
Department of Pediatric Pulmonology and Allergology, Erasmus Medical Centre-Sophia Children's Hospital, Rotterdam, the Netherlands.|Department of Radiology, Erasmus Medical Centre, Rotterdam, the Netherlands.
Pierluigi
P
Ciet
Centre for Imaging Sciences, The University of Manchester, Manchester, UK.|Biomedical Imaging Institute, The University of Manchester, Manchester, UK.|Bioxydyn Limited, Manchester, UK.
Geoffrey J M
GJ
Parker
Department of Biometry, Hannover Medical School, Hannover, Germany.
Armin
A
Koch
Institute of Diagnostic and Interventional Radiology, Hannover Medical School, Hannover, Germany.
Jens
J
Vogel-Claussen
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology*; D018450:Disease Progression; D006801:Humans; D007249:Inflammation / Q000503:physiopathology*; D008168:Lung / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D008991:Monitoring, Physiologic / Q000379:methods; D010100:Oxygen / Q000737:chemistry; D010477:Perfusion; D012119:Respiration; D012141:Respiratory Tract Infections / Q000150:complications / Q000503:physiopathology*; D013147:Spirometry; D016896:Treatment Outcome; D055815:Young Adult
D010100:Oxygen
lung disease; magnetic resonance imaging; pathobiology