Long-term glucocorticoid effect on bone mineral density in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Introduction Patients with 21-hydroxylase deficiency (21OHD) assume a lifelong glucocorticoid (GC) therapy. Excessive GC treatment increases the risk of osteoporosis and bone fractures, even though the role of substitutive therapy is not fully established: we analyzed the effect of GC dose on bone m...

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Published in:European journal of endocrinology 2016-08, Vol.175 (2), p.101-106
Main Authors: Ceccato, Filippo, Barbot, Mattia, Albiger, Nora, Zilio, Marialuisa, De Toni, Pietro, Luisetto, Giovanni, Zaninotto, Martina, Greggio, Nella Augusta, Boscaro, Marco, Scaroni, Carla, Camozzi, Valentina
Format: Article
Language:English
Subjects:
Absorptiometry, Photon
Adrenal Hyperplasia, Congenital - drug therapy
Adult
Bone Density - drug effects
Clinical Study
Dexamethasone - adverse effects
Dexamethasone - pharmacology
Dexamethasone - therapeutic use
Female
Femur Neck - diagnostic imaging
Femur Neck - drug effects
Fractures, Bone - chemically induced
Fractures, Bone - diagnostic imaging
Glucocorticoids - adverse effects
Glucocorticoids - pharmacology
Glucocorticoids - therapeutic use
Humans
Hydrocortisone - adverse effects
Hydrocortisone - pharmacology
Hydrocortisone - therapeutic use
Lumbar Vertebrae - diagnostic imaging
Lumbar Vertebrae - drug effects
Male
Middle Aged
Osteoporosis - chemically induced
Osteoporosis - diagnostic imaging
Prednisolone - adverse effects
Prednisolone - pharmacology
Prednisolone - therapeutic use
Young Adult
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Summary:Introduction Patients with 21-hydroxylase deficiency (21OHD) assume a lifelong glucocorticoid (GC) therapy. Excessive GC treatment increases the risk of osteoporosis and bone fractures, even though the role of substitutive therapy is not fully established: we analyzed the effect of GC dose on bone metabolism and bone mineral density (BMD) over time in patients with 21OHD. Methods We studied bone metabolism markers and BMD in 38 adult patients with 21OHD (19–47 years, 24 females and 14 males) and 38 matched healthy control. In 15 patients, BMD data were available at both baseline and after a long-term follow-up. Results BMD was lower in patients than in controls at lumbar spine (0.961±0.1g/cm2 vs 1.02±0.113g/cm2, P=0.014) and femur neck (0.736±0.128g/cm2 vs 0.828±0.103g/cm2, P=0.02); otherwise, after height correction, only femoral neck BMD was lower in patients (0.458±0.081g/cm2 vs 0.498±0.063g/cm2, P=0.028). In those 21OHD subjects with at least 10 years follow-up, we observed an increase in lumbar BMD (P=0.0429) and a decrease in femur neck BMD values (P=0.004). Cumulative GC dose was not related to bone metabolism or BMD. No patient experienced clinical fragility fractures. Conclusions BMD values are decreased in patients with 21OHD, which are in part explained by decreased height, but not by the dose of glucocorticoids. Nevertheless, bone status should be carefully monitored in patients with 21OHD.
ISSN:0804-4643
1479-683X
DOI:10.1530/EJE-16-0104