Poor compliance and increased mortality, depression and healthcare costs in patients with congenital adrenal hyperplasia

Objectives: To evaluate the risks of depression and all-cause mortality, healthcare utilisation costs and treatment adherence in congenital adrenal hyperplasia (CAH) in the United Kingdom. Design and methods: A retrospective, matched-cohort study using UK primary-care data from the Clinical Practice Research Datalink linked to hospital and death certification data. Patients diagnosed with CAH and having >= 1 corticosteroid prescription were matched 1: 10 to reference subjects. Risk of death and lifetime prevalence of depression were compared using Cox regression models. Direct financial costs were estimated for healthcare contacts. Treatment adherence was measured by medical possession ratio (MPR). Results: 605 patients with CAH were identified; 562 were matched. 270 CAH patients (2700 controls) were linkable to death-certificate data, with adjusted hazard ratio for all-cause mortality 5.17 (95% CI 2.81-9.50). Mean (S.D.)age at death in CAH patients was 54.8 (23.9) vs 72.8 (18.0) years in control patients. The prevalence ratio of depression in CAH vs control patients was 1.28 (95% CI 1.13-1.45). Mean (S.D.) annual healthcare costs were higher in CAH than controls: at age 0-6 years, 7038 pound (14 pound 846) vs 2879 pound (13 pound 972, P < 0.001); 7-17 years, 3766 pound (7494) pound vs 1232 pound (2451 pound, P < 0.001); 18-40 years, 1539 pound (872) pound vs 1344 pound (1620 pound, P = 0.007) and = 41 years, pound 4204 ( pound 4863) vs 1651 pound (2303 pound, P < 0.001). Treatment adherence was lowest in adults, with 141 (36%) of 396 eligible patients having an MPR < 80%. Conclusions: This first analysis of CAH in routine UK healthcare suggests that patients with CAH have increased mortality, depression and healthcare utilisation and low treatment adherence.