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Autoimmunity predominates in a large South African cohort with addison’s disease of mainly European descent despite long-standing disease and is associated with HLA DQB*0201

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Summary Objective 

We sought to determine whether autoimmunity is the predominant cause of Addison’s disease in South Africa and whether human leucocyte antigen (HLA) DQ association exists. Design 

We compiled a national registry of patients from primary care, referral centres and private practices. Patients 

A total of 144 patients, 94 of European descent, 34 Mixed Ancestry, 5 Asian and 11 Black Africans (mean age 45·9 years, range 2·7–88 years; mean duration of disease 13·1 years, range 0–50 years) and controls were matched for gender and ethnicity. All potential causes were investigated. Results 

Fifty one per cent of cases (74 patients) were autoimmune in aetiology. Either 21-hydroxylase autoantibodies (72 patients, 50% of entire patient group) or adrenocortical autoantibodies (35 patients, 24%) were present, while 23% of patients had both. None of the Asian (n = 5) or Black (n = 11) patients had evidence of autoimmune disease. Overall 8% of patients had tuberculosis, 4% adrenoleucodystrophy, 1% adrenocorticotrophic hormone resistance syndrome and 6% X-linked adrenal hypoplasia. In those with autoimmune disease primary hypothyroidism (47%), premature ovarian failure (8%) and type 1 diabetes (7%) were the most prevalent accompanying autoimmune conditions. HLA DQB1*0201 alleles predominated in the autoimmune group (DQB1*0201: 65%vs 43% of controls P = 0·017) with the *0201/*0302 heterozygous genotype being the most prevalent (28%vs 8%P = 0·02). Conclusions 

While autoimmunity accounts for at least half of patients with Addison’s disease in South Africa and is associated with HLA DQB1*0201, none of the Black Africans or Asians in this cohort had adrenal autoantibodies. Moreover, 21-hydroxylase autoantibodies were detectable in a higher proportion than adrenocortical autoantibodies, especially in those patients with a long history after disease onset.
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Document Type: Research Article

Affiliations: 1: Division of Endocrinology, University of Cape Town, Observatory, Cape Town, South Africa 2: Department of Public Health, University of Cape Town, Observatory, Cape Town, South Africa 3: Endocrinology Department, Christchurch Hospital and University of Otago, Christchurch, New Zealand 4: Nelson Mandela School of Medicine, University of KwaZulu-Natal, South Africa 5: Division of Medicine, University of Uppsala, Uppsala, Sweden 6: School for Biochemistry, Northwest University, Potchefstroom, South Africa 7: Centre for Biotechnology and Genomic Medicine, Medical College of Georgia, Augusta, GA, USA 8: Department of Pathology, Immunology and Laboratory Medicine and Paediatrics, Gainesville, FL, USA 9: Department of Paediatrics, University of Florida, Gainesville, FL, USA

Publication date: September 1, 2010

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