Abstract
BackgroundAddison’s disease is a potentially life-threatening disorder, and prompt diagnosis, and introduction of steroid replacement has resulted in near normal life-expectancy. There are limited data describing the clinical presentation of Addison’s disease in South Africa. It is hypothesised that patients may present in advanced state of ill-health, compared to Western countries.PatientsA national database of patients was compiled from primary care, referral centres and private practices. 148 patients were enrolled (97 white, 34 mixed ancestry, 5 Asian and 12 black).MethodsDemographic and clinical data were elicited using questionnaires. Biochemical data were obtained from folder reviews and laboratory archived results.ResultsThe majority of the cohort was women (62%). The median and inter-quartile age range (IQR) of patients at enrolment was 46.0 (32.0–61.0) years, with a wide range from 2.8–88.0 years. The median and IQR age at initial diagnosis was 34.0 (20.0–45.0) years (range 0.02–77.0) years, indicating that at the time of enrolment, the patients, on average, were diagnosed with Addison’s disease 12 years previously. Hyperpigmentation was observed in 76%, nausea and vomiting occurred in more than 40%, and weight loss was noted in 25%. Loss of consciousness as a presenting feature was recorded in 20%. with a 95% confidence interval [CI] of (14–28%) and shock occurred in 5% CI (1.5–8.5%). Case-finding was recorded at 3.1 per million.ConclusionsThe usual constellation of hyperpigmentation, nausea, vomiting and weight loss suggests Addison’s disease, but a significant proportion present with an advanced state of ill-health and Addisonian crises. A lower prevalence rate, compared to Western countries is suggested.
Highlights
South Africa has a population of 48 million people
Loss of consciousness as a presenting feature was recorded in 20%. with a 95% confidence interval [confidence intervals (CI)] of (14–28%) and shock occurred in 5% CI (1.5–8.5%)
Three patients declined to participate, citing personal reasons and a further three patients were too late to be enrolled in this observational study
Summary
South Africa has a population of 48 million people. In the absence of national insurance there is an unequal access to healthcare, which consists of a vast public health sector and a small, but expanding private health sector [1]. Addison’s disease (primary adrenal insufficiency) results from the destruction of the adrenal cortex, leading to decreased cortisol and aldosterone production. These hormones are vital for survival during stressful situations including infection [2]. This disorder is highly treatable and warrants prompt recognition. The described prevalence in Western countries is 39 to 144 per million [3,4,5]. There are limited data describing the clinical presentation of Addison’s disease in South Africa. It is hypothesised that patients may present in advanced state of ill-health, compared to Western countries. Patients: A national database of patients was compiled from primary care, referral centres and private practices. 148 patients were enrolled (97 white, 34 mixed ancestry, 5 Asian and 12 black)
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