Secondary adrenal insufficiency

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Adrenal insufficiency
Adrenal gland
ICD-10 E27.1-E27.4
ICD-9 255.4
MeSH D000309

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: hypocortisolism; hypocorticism; adrenocortical hypofunction

Overview

Adrenal insufficiency is a condition in which the adrenal glands, located above the kidneys, do not produce adequate amounts of steroid hormones (chemicals produced by the body that regulate organ function), primarily cortisol, but may also include impaired aldosterone production (a mineralocorticoid) which regulates sodium, potassium and water retention.[1][2] Craving for salt or salty foods due to the urinary losses of sodium is common.[3]

Addison's disease and congenital adrenal hyperplasia can manifest as adrenal insufficiency. If not treated, adrenal insufficiency may result in severe abdominal pains, diarrhea, vomiting, profound muscle weakness and fatigue, depression, extremely low blood pressure (hypotension), weight loss, kidney failure, changes in mood and personality, and shock (adrenal crisis).[4] An adrenal crisis often occurs if the body is subjected to stress, such as an accident, injury, surgery, or severe infection; death may quickly follow.[4]

Adrenal insufficiency can also occur when the hypothalamus or the pituitary gland, both located at the base of the skull, does not make adequate amounts of the hormones that assist in regulating adrenal function.[1][5][6] This is called secondary adrenal insufficiency and is caused by lack of production of ACTH in the pituitary or lack of CRH in the hypothalamus.[7]

Classification

There are three major types of adrenal insufficiency.

  • Primary adrenal insufficiency is due to impairment of the adrenal glands.
  • Secondary adrenal insufficiency is caused by impairment of the pituitary gland or hypothalamus.[8] The most common cause in the United States is exogenous steroid use. Other causes are a pituitary adenoma or microadenoma, hypothalamic tumor (surgical removal of a pituitary tumor can also suppress production of ACTH and lead to adrenal deficiency); Sheehan's syndrome, which is associated with impairment of only the pituitary gland.
  • Tertiary adrenal insufficiency is due to hypothalamic disease and decrease in corticotropin releasing factor (CRF).[9]

Causes

Common Causes

Causes by Organ System

Cardiovascular Arteritis, Hypotension, Hemorrhage, Infarction
Chemical / poisoning No underlying causes
Dermatologic POEMS syndrome
Drug Side Effect Aminoglutethimide, Corticosteroid withdrawal, Prednisone, Prednisolone, Dexamethasone, Trilostane
Ear Nose Throat No underlying causes
Endocrine Allgrove syndrome, Achalasia-addisonian syndrome, Adrenal aplasia / hypoplasia, Adrenal metastases, After surgery of cortisol-secreting tumor, Autoimmune polyendocrine syndrome type 2, Bilateral adrenalectomy , Congenital adrenal hyperplasia, Congenital adrenal hyperplasia, Glucocorticoid deficiency 1, IMAGE syndrome, POEMS syndrome, X-linked adrenal hypoplasia congenita
Environmental No underlying causes
Gastroenterologic Allgrove syndrome, Achalasia-addisonian syndrome, Hemochromatosis
Genetic Congenital adrenal hyperplasia, Cytochrome P450 oxidoreductase deficiency, Hereditary ACTH resistance, IMAGE syndrome, X-linked adrenal hypoplasia congenita
Hematologic Anticoagulation, Coagulopathy , Embolus, Leukemia, Lymphoma , Thrombosis
Iatrogenic Iatrogenic, Radiation therapy
Infectious Disease AIDS, Blastomycosis, CMV, Coccidiomycosis, Cryptococcosis, Histoplasmosis , Mycobacterium avium intracellulaire (MAI), Sepsis, Syphilis, Toxoplasmosis, Tuberculosis, Waterhouse-Friderichson syndrome
Musculoskeletal / Ortho IMAGE syndrome
Neurologic Adrenoleukodystrophy, Coma, Craniopharyngioma, Panhypopituitarism, POEMS syndrome
Nutritional / Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic Craniopharyngioma, Kaposi's sarcoma, Leukemia, Lymphoma
Opthalmologic No underlying causes
Overdose / Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Sarcoidosis,
Renal / Electrolyte Uremia, Waterhouse-Friderichson syndrome
Rheum / Immune / Allergy Autoimmune, Autoimmune polyendocrine syndrome type 2, Sarcoidosis
Sexual No underlying causes
Trauma Trauma
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Amyloidosis, Idiopathic, Iatrogenic, Radiation therapy, Surgery, Surgical removal of pituitary, Surgical removal of hypothalamus

Causes in Alphabetical Order

Diagnosis

Symptoms

There is often the slow insidious onset of the following symptoms (in alphabetical order):

Physical Examination

Vital Signs

Skin

  • There can be tanning of the skin. Tanning of the skin that may be patchy or even all over the body. Characteristic sites of tanning are skin creases (e.g. of the hands) and the inside of the cheek (buccal mucosa).
  • Vitiligo may also be present.[4]

Neck

Goitre may be present

Laboratory Findings

ACTH Stimulation Test

If the person is in adrenal crisis, the ACTH stimulation test[10] may be given. If not in crisis, cortisol, ACTH, aldosterone, renin, potassium and sodium are tested from a blood sample before the decision is made if the ACTH stimulation test needs to be performed. X-rays or CT of the adrenals may also be done.[1] The best test for adrenal insufficiency of autoimmune origin, representing more than ninety percent of all cases in a Western population, is measurement of 21-hydroxylase autoantibodies.

Source of pathology CRH ACTH DHEA DHEA-S cortisol aldosterone renin Na K Causes5
hypothalamus
(tertiary)1
low low low low low3 low low low low tumor of the hypothalamus (adenoma), antibodies, environment (i.e. toxins), head injury
pituitary
(secondary)
high2 low low low low3 low low low low tumor of the pituitary (adenoma), antibodies, environment, head injury,
surgical removal6, Sheehan's syndrome
adrenal glands
(primary)7
high high high high low4 low high low high tumor of the adrenal (adenoma), stress, antibodies, environment, Addison's Disease, trauma, surgical removal (resection), miliary tuberculosis of the adrenal
1 Automatically includes diagnosis of secondary (hypopituitarism)
2 Only if CRH production in the hypothalamus is intact
3 Value doubles or more in stimulation
4 Value less than doubles in stimulation
5 Most common, does not include all possible causes
6 Usually because of very large tumor (macroadenoma)
7 Includes Addison's disease

Treatment

  • Adrenal crisis
    • Intravenous fluids[4]
    • Intravenous steroid (Solu-Cortef/injectable hydrocortisone) later hydrocortisone, prednisone or methylpredisolone tablets[4]
    • Rest
  • Mineralocorticoid deficiency (low aldosterone)
    • Fludrocortisone Acetate

(To balance sodium, potassium and increase water retention)[4]

See also

References

  1. 1.0 1.1 1.2 Eileen K. Corrigan (2007). "Adrenal Insufficiency (Secondary Addison's or Addison's Disease)". NIH Publication No. 90-3054.
  2. Adrenal+Insufficiency at the US National Library of Medicine Medical Subject Headings (MeSH)
  3. Ten S, New M, Maclaren N (2001). "Clinical review 130: Addison's disease 2001". J. Clin. Endocrinol. Metab. 86 (7): 2909–22. doi:10.1210/jc.86.7.2909. PMID 11443143.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 Ashley B. Grossman, MD (2007). "Addison's Disease". Adrenal Gland Disorders.
  5. Brender E, Lynm C, Glass RM (2005). "JAMA patient page. Adrenal insufficiency". JAMA. 294 (19): 2528. doi:10.1001/jama.294.19.2528. PMID 16287965.
  6. "Dorlands Medical Dictionary:adrenal insufficiency".
  7. "Secondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional".
  8. "hypopituitary". 2006.
  9. http://www.endotext.org/adrenal/adrenal13/adrenal13.htm
  10. Henzen C (2011). "[Adrenal insufficiency--diagnosis and treatment in clinical practice]". Ther Umsch. 68 (6): 337–43. doi:10.1024/0040-5930/a000174. PMID 21656493. Unknown parameter |month= ignored (help)

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