Endocrinol Metab.  2017 Dec;32(4):466-474. 10.3803/EnM.2017.32.4.466.

Characteristics of Korean Patients with Primary Adrenal Insufficiency: A Registry-Based Nationwide Survey in Korea

• Hong AR
• Ryu OH
• Kim SY
• Kim SW
• The Korean Adrenal Gland and Endocrine Hypertension Study Group
• Korean Endocrine Society

• KMID: 2406191
• DOI: http://doi.org/10.3803/EnM.2017.32.4.466

Abstract

BACKGROUND
Primary adrenal insufficiency (PAI) is a rare, potentially life-threatening condition. There are few Korean studies on PAI, and most have had small sample sizes. We aimed to examine the etiology, clinical characteristics, treatment, and mortality of PAI in Korean patients.
METHODS
A nationwide, multicenter, registry-based survey was conducted to identify adults diagnosed with or treated for PAI at 30 secondary or tertiary care institutions in Korea between 2000 and 2014.
RESULTS
A total of 269 patients with PAI were identified. The prevalence of PAI was 4.17 per million. The estimated incidence was 0.45 per million per year. The mean age at diagnosis was 49.0 years, and PAI was more prevalent in men. Adrenal tuberculosis was the most common cause of PAI in patients diagnosed before 2000; for those diagnosed thereafter, adrenal metastasis and tuberculosis were comparable leading causes. The etiology of PAI was not identified in 34.9% of cases. Of the patients receiving glucocorticoid replacement therapy, prednisolone was more frequently administered than hydrocortisone (69.4% vs. 26.5%, respectively), and only 27.1% of all patients received fludrocortisone. We observed an increased prevalence of metabolic disease and osteoporosis during the follow-up period (median, 60.2 months). The observed overall mortality and disease-specific mortality rates were 11.9% and 3.1%, respectively.
CONCLUSION
The prevalence of PAI is significantly lower in Koreans than in reports from Western countries. The high frequency undetermined etiology in patients with PAI suggests the need to reveal accurate etiology of PAI in Korea.

Keyword

Addison disease; Autoimmune diseases; Glucocorticoids; Mortality

MeSH Terms

Addison Disease*
Adult
Autoimmune Diseases
Diagnosis
Fludrocortisone
Follow-Up Studies
Glucocorticoids
Humans
Hydrocortisone
Incidence
Korea*
Male
Metabolic Diseases
Mortality
Neoplasm Metastasis
Osteoporosis
Prednisolone
Prevalence
Sample Size
Tertiary Healthcare
Tuberculosis
Fludrocortisone
Glucocorticoids
Hydrocortisone
Prednisolone

Figure

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  Fig. 1 Observed incidence of primary adrenal insufficiency in Korea over time.

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  Fig. 2 Geographic distribution of patients with primary adrenal insufficiency in Korea.

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  Fig. 3 Primary adrenal insufficiency etiologic pattern in patients diagnosed before 2000 and between 2000 and 2014. Tbc, tuberculosis; ALDS, adrenoleukodystrophy; PGA, polyglandular autoimmune syndrome; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.

• 

  Fig. 4 Prevalence of comorbidities at diagnosis and during follow-up in patients with primary adrenal insufficiency. aP<0.05.

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