Unexpected

Vertebral Fractures
in Adults with Cystic
Fibrosis

Aoife Lynam
Adult CF Dietitian
Wessex Adult CF Centre U.K.
 Aim
• Determine prevalence of vertebral fractures
(VF)
• Describe the characteristics of those with VF
 Wessex Adult CF Bone Service
• Achieve normal weight for height
• Aim to maximise fat free mass
• Adequate Calcium intake
• Weight bearing exercise
• Advice against smoking and excessive alcohol
• DEXA with IVA
• Bone turnover markers (CTX and P1NP)
• Vitamin D and PTH
• Fracture history
 Vertebral Fractures
• 5 times risk of spinal fracture
• 2 times risk for hip fracture
• Thoracic vertebral fractures
– inhibit airway clearance
– ability to receive physiotherapy
– contraindicate transplant
• What do the guidelines say?
European CF Guidelines
 Method
• Retrospective review of DEXA
• All Instant Vertebral Assessments (IVAs)
reviewed for VF
• VFs discussed with Consultant Rheumatologist
• Further investigations discussed with
Consultant Radiologist
• Data collected and analysed with Microsoft
Excel
Results

280 DEXA

266 IVA
95% of those who
had a DEXA

32 VF
12% of those who
had IVA
 Results
• Median age of those with VF was 39 years
(IQR 21.5–51.3 years)
– 13/32 (41%) aged ≤30 years
• VF was largely silent
– 4/32 individual (13%) reported pain
• Males accounted for 69% of those with VF (p <
0.05)
• 27% pancreatic sufficient
– 16% total patient group PS
 Results Continued
• BMD not a predictor of fracture risk

27%
33%

40%

– Comparison of BMD in those with VF with those without

VF revealed no difference (p=0.5)
 Results Continued
• BMI not a predictor of fracture risk
– Mean BMI of those with a VF (25.05kg/m²) vs.
those with no VF (23.1kg/m²) (p = 0.45)
• Severe lung disease (Fev1 <40%) not a
predictor of fracture risk
– Severe lung disease in VF (22%) vs. those with no
VF (20%) (p=0.22)
 Results Continued
• Two or more fractures identified in
11/32 (34%)
• Majority (28/32, 91%) of VFs in
thoracic spine
– lumbar spine (8/32, 25%)
• 9 underwent further investigation
– MRI (n=7)
– lateral spinal XR (n=2)
• 4 cases of confirmed Scheuermann’s
disease
 Scheuermann’s Disease
• Growth disturbance thoracic spine characterised by rigid
hyperkyphosis caused by wedge-shaped vertebrae3.
• Minimum 3 consecutive vertebral bodies with a minimum of
5° of wedging4
• Incidence range from 0.4 to 8%
• The aetiology is unknown
– major genetic component
– mechanical
– hormonal
– pathogenesis likely multifactorial5,6
 Discussion
• Pooled prevalence of VF in CF was
14%6
• Majority of VFs were in the thoracic
spine7
• BMD was significantly higher
amongst those with VFs7
• VFs more prevalent amongst males8
 Conclusion
• VF strong predictor of future spine and hip fractures
• Screening
• Accurate diagnosis
• Unambiguous reporting
• Asymptomatic in 86% of cases
– BMI
– BMD
– Lung disease severity
• IVA assessment at DEXA is recommended
– No additional time
– No additional expense
• Potential link between Scheuermann’s disease and CF
Thank you

Questions?
 References
1. Van der Velde, R.Y. et al., 2018. Incidence of subsequent fractures in the UK between 1990
and 2012 among individuals 50 years or older. Osteoporosis International. 29, pp. 2469-
2475.
2. Orens J.B., et al., 2006. International Guidelines for the Selection of Lung Transplant
Candidates: 2006 Update-A Consensus Report From the Pulmonary Scientific Council of the
International Society for Heart and Lung Transplantation. J Hear Lung Transplant.25(7) pp.
745–55.
3. Scheuermann, H., 1920. Kyfosis dorsalis juvenilis. Ugeskr Laeger. 82(12) pp. 385–393.
4. Sorensen K (1964) Scheuermann’s juvenile kyphosis: clinical appearances, radiography,
aetiology, and prognosis. Munksgaard, Copenhagen
5. Holick, M.F., 2009. Vitamin D status: measurement, interpretation and clinical application.
Annal Epidemiology, 19(2), pp. 73–78.
6. Paccou J., et al., 2010 The prevalence of osteoporosis, osteopenia, and fractures among
adults with cystic fibrosis: A systematic literature review with meta-analysis. Calcified
Tissue International. 86(1) pp. 1–7.
7. Stephenson, A., et al., 2006. Prevalence of vertebral fractures in adults with cystic fibrosis
and their relationship to bone mineral density. Chest. 130, pp. 539-544
8. Rosinni, M., et al., 2004. Prevalence and correlates of vertebral fractures in adults with
cystic fibrosis. Bone. 35 pp. 771-776.
9. Sermet-Gaudelus, I. et al., 2011. European cystic fibrosis bone mineralisation guidelines.
Journal of Cystic Fibrosis, (10)2, pp. S16–S23.