Body composition, lung function, and prevalent and progressive bone deficits among adults with cystic fibrosis

Abstract

Introduction: Cystic fibrosis (CF) is associated with osteoporosis and incident fracture. This study assessed independent predictors of baseline and 2-year changes in bone mineral density (BMD) in adults with CF.

Methods: Sixty-four adult patients with CF, ages 18-57, were recruited from the Massachusetts General Hospital Cystic Fibrosis Care Center. Dual-energy X-ray absorptiometry (DXA) was performed at the spine and radius at baseline and 2 years (in 39 subjects). Estimates of fat-free mass index (FFMI) and fat mass index (FMI) were determined using height, weight, and tetrapolar bioelectric impedance analysis. All subjects completed lung spirometry within 1 month of the study visit. Linear regression models evaluated predictors of baseline BMD Z-scores and change in PA spine BMD Z-score over 2 years. Two definitions of low BMD were studied based on Z-score (≤-1.0 and ≤-2.0).

Results: Low BMD was present in 52% of subjects. Subjects with low BMD were more likely to be male (67% vs. 32%, P=0.009), were more likely to be currently using glucocorticoids (21% vs. 0%, P<0.001), had lower percent body fat (P=0.04), and were more likely to have had a previous fracture (60% vs. 46%, P=0.007). In multivariable models, greater FFMI and height, but not greater FMI, were associated with greater BMD. In multivariable models, low forced vital capacity (FVC) and greater FMI were associated with greater loss of BMD at the PA spine over two years.

Conclusions: Male sex, short stature, and low lean mass are associated with low BMD in CF. Greater adiposity and lower lung function are predictors of negative change in BMD Z-score over 2 years.

Keywords: Bone mineral density; Cystic fibrosis; Dual-energy X-ray absorptiometry; Fat mass; Fat-free mass; Pulmonary function test.