Putting lung function and physiology into perspective: cystic fibrosis in adults

Horsley, Alex; Siddiqui, Salman
Respirology, January 2015, Vol. 20 Issue: Number 1 p33-45, 13p
Abstract: Adult cystic fibrosis (CF) is notable for the wide heterogeneity in severity
of disease expression, both between patients and within the lungs of
individuals. Although CFairways disease appears to start in the small
airways, in adults there is typically widespread bronchiectasis,
increased airway secretions, and extensive obstruction and inflammation
of the small airways. The complexity and heterogeneity of airways
disease in CFmeans that although there are many different methods of
assessing and describing lung ‘function’, none of these
single‐dimensional tests is able to provide a comprehensive assessment
of lung physiology across the spectrum seen in adult CF. The most
widely described measure, the forced expiratory volume in 1 s, remains
a useful and simple clinical tool, but is insensitive to early changes
and may be dissociated from other more detailed assessments of disease
severity such as computed tomography. In this review, we also discuss
the use of more sensitive novel assessments such as multiple breath
washout tests and impulse oscillometry, as well as the role of
cardiopulmonary exercise testing. In the future, hyperpolarized gas
magnetic resonance imaging techniques that combine regional structural
and functional information may help us to better understand these
measures, their applications and limitations.;