Abstract

ObjectiveWe wished to determine the prevalence, etiology, presentation, and available management strategies for primary adrenal insufficiency (PAI) in South Africa (SA), hypothesizing a prevalence greater than the described 3.1 per million. There is great inequity in healthcare allocation, as two parallel healthcare systems exist, potentially modifying PAI patients’ clinical profiles, private being better resourced than public healthcare.MethodsAn online survey of physicians’ experience relating to PAI.ResultsThe physicians were managing 811 patients, equal to a prevalence of 14.2 per million. Likely causes of PAI in public/ academic vs private settings included: AIDS-related [304 (44.8%) vs 5 (3.8%); p<0.001], tuberculosis [288 (42.5%) vs 8 (6.0%); p<0.001], autoimmune disease [50 (7.4%) vs 88 (66.2%); p<0.001], malignancy [27 (4.0%) vs 7 (5.3%); p = 0.500], genetic including adrenoleukodystrophy (ALD) [5 (0.7%) vs 16 (12.0%); p<0.001], respectively. Overall, more patients presented with nausea [101 (74.3%) and vomiting 89 (65.9%), than diarrhoea 76 (58.9%); p = 0.008 and 126 (15.5%) in adrenal crisis. Features suggestive of a crisis were hypoglycaemia [40 (78.4%) vs 42 (48.8%); p = 0.001], shock [36 (67.9%) vs 31(36.9%); p<0.001], and loss of consciousness [25 (52.1%) vs 27 (32.9%); p = 0.031]. Greater unavailability of antibody testing in the public vs. the private sector [32 (66.7%) vs 30 (32.1%); p = 0.001], [serum-ACTH 25 (52.1%) vs 16 (19.5%); p<0.001] and glucocorticoids were [26 (54.2%) vs 33 (40.2%); p = 0.015]. Many patients, 389(66.7%) were not using identification, indicating that they need steroids in an emergency.ConclusionA survey of South African physicians suggests a higher prevalence than previously reported. Patients presented with typical symptoms, and 15.5% presented in adrenal crisis. Significant disparities in the availability of physicians’ expertise, diagnostic resources, and management options were noted in the public versus private settings. Greater awareness among health practitioners to timeously diagnose PAI is required to prevent a life-threatening outcome.

Highlights

  • Patients presented with typical symptoms, and 15.5% presented in adrenal crisis

  • We previously identified the significant barriers to diagnosis and management of primary adrenal insufficiency (PAI) in Africa and that the etiology was predominantly related to tuberculosis (TB) and human immune deficiency virus (HIV) [1]

  • Opportunistic infections (OIs) in Acquired Immune Deficiency Syndrome (AIDS) have been implicated in PAI development [12,13,14], yet the real impact of these OIs causing PAI has never been assessed in any large study

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Summary

Introduction

We previously identified the significant barriers to diagnosis and management of primary adrenal insufficiency (PAI) in Africa and that the etiology was predominantly related to tuberculosis (TB) and human immune deficiency virus (HIV) [1]. The underlying etiology has evolved from predominantly TB to autoimmune, especially in populations of European descent [6,7]. In a descriptive study of acute PAI in SA in 1999, before the peak incidence of Acquired Immune Deficiency Syndrome (AIDS) in SA, the underlying etiology was considered idiopathic in 42%, associated with active TB (18%), previous TB (16%), autoimmune (12%) and metastases in 6% [9]. Given the high prevalence of AIDS, there is likely a higher proportion of PAI patients attributable to HIV infection than previously thought

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