Endocrine Abstracts

Background: Quality of life (QoL) has been variously reported as normal or impaired in congenital adrenal hyperplasia (CAH) adults. We found impaired QoL in UK CAH adults and now report the relationship between QoL, glucocorticoid treatment and health outcomes in these patients.

Methods: Cross-sectional analysis of 151 CAH adults with 21-hydroxylase deficiency aged 18–69 years in whom QoL (SF-36), glucocorticoid regimen, anthropometric, and metabolic measures were recorded. Relationships were examined between QoL, type of glucocorticoid (hydrocortisone, prednisolone, hydrocortisone plus prednisolone and any regimen with dexamethasone), and dose of glucocorticoid expressed as prednisolone dose equivalent, PreDDefault (<5, 5–7.4, ≥7.5 mg/day). Principal components analysis (PCA) was undertaken to identify clusters of associated clinical and biochemical features and the principal component (PC) scores used in regression analysis as predictor of QoL.

Results: There was a difference in QoL according to glucocorticoid treatment regimen for vitality (ANOVA: F_3,144=5.1, P=0.002) and mental health (F_3,144=3.9, P=0.011). In post hoc analysis, vitality and mental health z-scores were higher in patients on hydrocortisone monotherapy compared with the other treatment groups (P<0.05). QoL did not relate to PreDEq or mutation severity. In PCA, three PCs were identified that explained 61% of the total variance (r²) in observed variables. Regression analysis demonstrated that PC2, reflecting adiposity and insulin resistance (waist circumference, serum triglycerides, HOMA-IR and HDL-cholesterol) related to QoL scores, specifically impaired physical function (β=−0.72, 95% CI: −1.11 to −0.34, r²=19.9%, P<0.001), bodily pain (β=−0.51, 95% CI: −0.77 to −0.23, r²=21.6%, P<0.001), general health (β=−0.50, 95% CI: −0.80 to −0.20, r²=16.0%, P=0.001) and vitality (β=−0.44, 95% CI: −0.65 to −0.16 r²=15.5%, P=0.002).

Conclusions: Increased adiposity and insulin resistance, and use of prednisolone or dexamethasone, are associated with impaired QoL in adults with CAH. Further studies are justified to establish whether optimising the choice of glucocorticoid treatment and/or weight loss can improve QoL in this disadvantaged patient group.

Acknowledgements

CaHASE are grateful to The Clinical Endocrinology Trust for their financial support and the Society for Endocrinology for management of the project.

Declaration of funding: The Clinical Endocrinology Trust (UK Registered Charity Number 288679) and the Society for Endocrinology.