Primary adrenal insufficiency - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Presentation of primary adrenal insufficiency (Addison disease) may be acute or insidious, with progressive substantial fatigue and generalized weakness associated with mucocutaneous hyperpigmentation, hypotension and/or postural hypotension, and salt craving.
Adrenocorticotropic hormone stimulation test is the definitive test to confirm or exclude the diagnosis of early primary adrenal insufficiency.
All patients require mineralocorticoid and glucocorticoid replacement therapy for life.
Use of a medical alert (bracelet or card) warning about their condition, and education on when to increase glucocorticoid replacement doses appropriately during surgery and during any stressful or infectious conditions, is vital to prevent potentially life-threatening adrenal crises.
Treatment complications arise from over-replacement of mineralocorticoid and/or glucocorticoid.

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History & Exam
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Generally hyperpigmented young man with primary adrenal insufficiency: exaggerated pigmentation over pressure points on elbows
Hands of patient with primary adrenal insufficiency showing: hyperpigmentation exaggerated on sun-exposed dorsal surface and creases; area of vitiligo in skin over left second metacarpophalangeal joint
Area of buccal hyperpigmentation where teeth pinch mucous membrane while chewing
Hands of patient with primary adrenal insufficiency showing: hyperpigmentation exaggerated on sun-exposed dorsal surface and creases; area of vitiligo in skin over left second metacarpophalangeal joint

Citations

Key Articles

Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and treatment of primary adrenal insufficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2016 Feb;101(2):364-89.[Abstract][Full Text]
Husebye ES, Pearce SH, Krone NP, et al. Adrenal insufficiency. Lancet. 2021 Feb 13;397(10274):613-29.[Abstract]
Betterle C, Presotto F, Furmaniak J. Epidemiology, pathogenesis, and diagnosis of Addison's disease in adults. J Endocrinol Invest. 2019 Dec;42(12):1407-33.[Abstract]
Arlt W; Society for Endocrinology Clinical Committee. Society For Endocrinology endocrine emergency guidance: emergency management of acute adrenal insufficiency (adrenal crisis) in adult patients. Endocr Connect. 2016 Sep;5(5):G1-3.[Abstract][Full Text]

Referenced Articles

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