Secondary adrenal insufficiency: Difference between revisions
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====CT Scan==== | ====CT Scan==== | ||
* [[CT]] scan findings in primary adrenal insufficiency include: | * [[CT]] scan findings in '''primary adrenal insufficiency''' include: | ||
:* Small adrenal remnants bilaterally | :* Small adrenal remnants bilaterally | ||
:* Dense calcification in one or both adrenal beds without evidence of normal glandular remnants - in [[TB]]<ref name="pmid7119194">{{cite journal |author=Doppman JL, Gill JR, Nienhuis AW, Earll JM, Long JA |title=CT findings in Addison's disease |journal=J Comput Assist Tomogr |volume=6 |issue=4 |pages=757–61 |year=1982 |month=August |pmid=7119194 |doi= |url=}}</ref> | :* Dense calcification in one or both adrenal beds without evidence of normal glandular remnants - in [[TB]]<ref name="pmid7119194">{{cite journal |author=Doppman JL, Gill JR, Nienhuis AW, Earll JM, Long JA |title=CT findings in Addison's disease |journal=J Comput Assist Tomogr |volume=6 |issue=4 |pages=757–61 |year=1982 |month=August |pmid=7119194 |doi= |url=}}</ref> | ||
:* Enlarged nonhomogeneous glands of normal contour - in [[Histoplasmosis]] | :* Enlarged nonhomogeneous glands of normal contour - in [[Histoplasmosis]] | ||
:* Atrophic adrenal glands - in [[idiopathic]] [[autoimmune]] Addison's disease. | :* Atrophic adrenal glands - in [[idiopathic]] [[autoimmune]] [[Addison's disease]]. | ||
* In '''secondary adrenal insufficiency''' the following findings may be seen on CT head: | |||
:* Calcified [[pituitary]] and/or [[hypothalamus]]. | |||
:* [[Pituitary adenomas]] - micro / macro | |||
:* Absent pituitary and/or hypothalamus - surgical removal | |||
==Treatment== | ==Treatment== | ||
Revision as of 14:54, 14 August 2012
| Adrenal insufficiency | |
 | |
|---|---|
| Adrenal gland | |
| ICD-10 | E27.1-E27.4 |
| ICD-9 | 255.4 |
| MeSH | D000309 |
For patient information click here
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]
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][4][5] 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.[6]
Classification
There are three major types of adrenal insufficiency.
- Primary adrenal insufficiency is due to impairment of the adrenal glands.
- One subtype is called idiopathic or unknown cause of adrenal insufficiency.
- 80% are due to an autoimmune disease called Addison's disease or autoimmune adrenalitis.
- Other cases are due to congenital adrenal hyperplasia or an adenoma (tumor) of the adrenal gland.
- Secondary adrenal insufficiency is caused by impairment of the pituitary gland or hypothalamus.[7] 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).[8]
Pathophysiology
- Primary adrenal insufficiency:
Here the defect lies in the adrenal glands. Autoantibodies, infections, drugs and mutations cause destruction of the adrenal glands leading to deficiency of adrenal hormones and thus causing symptoms.
- Secondary adrenal insufficiency:
ACTH deficiency caused by diseases of the pituitary gland is the main cause for adrenal insufficiency. ACTH deficiency leads to loss of stimulus for the production of adrenal hormones. ACTH levels in the blood can differentiate primary adrenal insufficiency from the secondary form. In the primary form the ACTH levels are high because of the loss of negative feedback from the adrenal hormones.
- Tertiary adrenal insufficiency:
The hypothalamus in the brain secretes a hormone called corticotropin releasing factor (CRF). This hormone provides the necessary stimulus to the pituitary for secreting ACTH which in turn activates the process of production of adrenal hormones. In diseases of the hypothalamus, the CRF is not produced leading to a decreased production of ACTH and in turn the adrenal hormones.
Causes
Common Causes
- Acute adrenal insufficiency
- Addison's disease (autoimmune adrenalitis)
- Septic shock
- Waterhouse-Friderichsen syndrome
- Addisonian crisis in case of:
- Discontinuing corticosteroid therapy without tapering the dosage
- Surgery, illness or any other form of stress in patients with long-term corticosteroid therapy
- Chronic adrenal insufficiency
Causes by Organ System
Causes in Alphabetical Order
Differentiating Adrenal insufficiency from other Diseases
Diseases with similar symptoms include:
Risk Factors
The following conditions increase the risk of adrenal insufficiency:
- Genetic defects of adrenal gland
- Underactive parathyroid glands
- Pernicious anemia
- Chronic fungal infections
- Chronic active hepatitis
- Diabetes
- Vitiligo
- Tuberculosis
- Amyloidosis
- AIDS-associated infections
- Cancer
Natural History, Complications and Prognosis
Complications
Complications of adrenal insufficiency include:
- Cardiac arrhythmias - due to hyperkalemia
- Psychosis
- Addisonian crisis
Prognosis
The prognosis of adrenal insufficiency depends on:
- Whether the patient has received hormone replacement or not.
- When stress happens on the patients with adrenal insufficiency, whether the treatment protocols are adjusted or not.
Diagnosis
Symptoms
There is often the slow insidious onset of the following symptoms:
- Dehydration
- Diarrhea
- Abdominal pain
- Disorientation
- Dizziness
- Muscle aches
- Nausea
- 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).
- Tiredness - fatigue
- Vomiting
- Weakness
- Weight loss
Physical Examination
Appearance
- The patient may be dehydrated and lethargic.
Vital Signs
- Low blood pressure that falls further when standing (orthostatic hypotension)
- Cardiovascular collapse may be present
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.
- Absence of axillary and pubic hair in females as a result of loss of adrenal androgens.
- Pallor may be present.
Neck
- Goitre may be present
Extremities
Neurologic
Laboratory Findings
ACTH Stimulation Test
If the person is in adrenal crisis, the ACTH stimulation test[25] 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 |
Routine Investigations
- Complete blood count and differential count - Eosinophilia and lymphocytosis (increased number of eosinophils or lymphocytes, two types of white blood cells). Normocytic anemia may be present.
- Serum ACTH levels - elevated in primary adrenal insufficiency and decreased in secondary form.
- Hypoglycemia - low blood sugar (worse in children)
- Serum electrolytes:
- Hyponatraemia - (low blood sodium levels)
- Hyperkalemia - (raised blood potassium levels), due to loss of production of the hormone aldosterone
- Mild hypercalcemia - in 20% patients
- Mild non–anion-gap metabolic acidosis
- Serum cortisol - decreased (<25mcg/dL)
- Urine and sweat sodium - elevated
- Elevated BUN and creatinine - when hypovolemia is the cause.
- Prolactin - mild elevation may be present.
Imaging
X-ray
- Chest X-ray may show the following findings:
- Size of the heart appears to be decreased.
- Infections either earlier or current (TB, fungal) can be diagnosed using chest x-ray when they are the cause of Addison's disease.
- X-ray exams of the abdomen may be taken to see if the adrenals have any signs of calcium deposits. Calcium deposits may indicate tuberculosis (TB)
CT Scan
- CT scan findings in primary adrenal insufficiency include:
- Small adrenal remnants bilaterally
- Dense calcification in one or both adrenal beds without evidence of normal glandular remnants - in TB[26]
- Enlarged nonhomogeneous glands of normal contour - in Histoplasmosis
- Atrophic adrenal glands - in idiopathic autoimmune Addison's disease.
- In secondary adrenal insufficiency the following findings may be seen on CT head:
- Calcified pituitary and/or hypothalamus.
- Pituitary adenomas - micro / macro
- Absent pituitary and/or hypothalamus - surgical removal
Treatment
- Adrenal crisis
- Intravenous fluids
- Intravenous steroid (Solu-Cortef/injectable hydrocortisone) later hydrocortisone, prednisone or methylpredisolone tablets
- Rest
- Cortisol deficiency (primary and secondary)
- Hydrocortisone (Cortef)
- Prednisone (Deltasone)
- Prednisolone (Delta-Cortef)
- Methylprednisolone (Medrol)
- Dexamethasone (Decadron)
- Mineralocorticoid deficiency (low aldosterone)
- Fludrocortisone acetate
(To balance sodium, potassium and increase water retention)
Prevention
Acute adrenal insufficiency is fatal. When patients with adrenal insufficiency experience the following stress, they must be very careful.
- Surgery: People who are not currently taking glucocorticoids but who have taken long-term glucocorticoids in the past year, and people with chronic adrenal insufficiency should tell their doctor before surgery. These patients must be treated with intravenous glucocorticoids and saline before surgery and continues until the patient is fully awake after surgery and able to take medication by mouth.
- Illness: Patients with adrenal insufficiency should know the essentiality of increasing medication during such periods of stress. During illness, oral dosing of glucocorticoid needs to be adjusted to mimic the normal response of the adrenal glands on the body. When significant fever or injury happen, the patients may require triple oral dosing. Once recovery from the stress event, doses can be returned to maintenance levels. Be careful, when the conditions of severe infections, vomiting, or diarrhea occur, these signs suggest the possibility of addisonian crisis.
- Pregnancy: If nausea and vomiting in early pregnant woman with adrenal insufficiency interfere with taking medication by mouth, injections of the hormone may be necessary. During delivery, treatment is similar to that of people needing surgery.
See also
- Addison disease, primary adrenocortical insufficiency
- ACTH stimulation test
- Cushing's syndrome, overproduction of cortisol
- Insulin tolerance test, another test used to identify sub-types of adrenal insufficiency.
- Adrenal fatigue (hypoadrenia), a term used in alternative medicine to describe a believed exhaustion of the adrenal glands.
References
- ↑ 1.0 1.1 1.2 Eileen K. Corrigan (2007). "Adrenal Insufficiency (Secondary Addison's or Addison's Disease)". NIH Publication No. 90-3054.
- ↑ Adrenal+Insufficiency at the US National Library of Medicine Medical Subject Headings (MeSH)
- ↑ 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.
- ↑ 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.
- ↑ "Dorlands Medical Dictionary:adrenal insufficiency".
- ↑ "Secondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional".
- ↑ "hypopituitary". 2006.
- ↑ http://www.endotext.org/adrenal/adrenal13/adrenal13.htm
- ↑ 9.0 9.1 Huebner A, Yoon SJ, Ozkinay F; et al. (2000). "Triple A syndrome--clinical aspects and molecular genetics". Endocr. Res. 26 (4): 751–9. PMID 11196451. Unknown parameter
|month=ignored (help) - ↑ Mosser J, Douar AM, Sarde CO; et al. (1993). "Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters". Nature. 361 (6414): 726–30. doi:10.1038/361726a0. PMID 8441467. Unknown parameter
|month=ignored (help) - ↑ Shashidhar PK, Shashikala GV (2012). "Low dose adrenocorticotropic hormone test and adrenal insufficiency in critically ill acquired immunodeficiency syndrome patients". Indian J Endocrinol Metab. 16 (3): 389–94. doi:10.4103/2230-8210.95680. PMC 3354846. PMID 22629505. Unknown parameter
|month=ignored (help) - ↑ Eledrisi MS, Verghese AC (2001). "Adrenal insufficiency in HIV infection: a review and recommendations". Am. J. Med. Sci. 321 (2): 137–44. PMID 11217816. Unknown parameter
|month=ignored (help) - ↑ Cocco C, Meloni A, Mariotti S; et al. (2012). "Novel neuronal and endocrine autoantibody targets in autoimmune polyendocrine syndrome type 1". Autoimmunity. 45 (6): 485–94. doi:10.3109/08916934.2012.680632. PMID 22506635. Unknown parameter
|month=ignored (help) - ↑ Osa SR, Peterson RE, Roberts RB (1981). "Recovery of adrenal reserve following treatment of disseminated South American blastomycosis". Am. J. Med. 71 (2): 298–301. PMID 6266250. Unknown parameter
|month=ignored (help) - ↑ Ardalan M, Shoja MM (2009). "Cytomegalovirus-induced adrenal insufficiency in a renal transplant recipient". Transplant. Proc. 41 (7): 2915–6. doi:10.1016/j.transproceed.2009.07.024. PMID 19765472. Unknown parameter
|month=ignored (help) - ↑ Mihalache A, Lamy O, Waeber G, Schneider A (2007). "[Adrenal insufficiency and hypercalcemia--an unusual presentation]". Praxis (Bern 1994) (in German). 96 (45): 1761–5. PMID 18050601. Unknown parameter
|month=ignored (help) - ↑ Kawamura M, Miyazaki S, Mashiko S; et al. (1998). "Disseminated cryptococcosis associated with adrenal masses and insufficiency". Am. J. Med. Sci. 316 (1): 60–4. PMID 9671046. Unknown parameter
|month=ignored (help) - ↑ Turan S, Hughes C, Atay Z; et al. (2012). "An atypical case of familial glucocorticoid deficiency without pigmentation caused by coexistent homozygous mutations in MC2R (T152K) and MC1R (R160W)". J. Clin. Endocrinol. Metab. 97 (5): E771–4. doi:10.1210/jc.2011-2414. PMC 3396854. PMID 22337906. Unknown parameter
|month=ignored (help) - ↑ Francke U, Harper JF, Darras BT; et al. (1987). "Congenital adrenal hypoplasia, myopathy, and glycerol kinase deficiency: molecular genetic evidence for deletions". Am. J. Hum. Genet. 40 (3): 212–27. PMC 1684111. PMID 2883886. Unknown parameter
|month=ignored (help) - ↑ Pedersen-Bjergaard U, Thorsteinsson B, Kirkegaard BC (1996). "[Pituitary function in hemochromatosis]". Ugeskr. Laeg. (in Danish). 158 (13): 1818–22. PMID 8650756. Unknown parameter
|month=ignored (help) - ↑ Pedreira CC, Savarirayan R, Zacharin MR (2004). "IMAGe syndrome: a complex disorder affecting growth, adrenal and gonadal function, and skeletal development". J. Pediatr. 144 (2): 274–7. doi:10.1016/j.jpeds.2003.09.052. PMID 14760276. Unknown parameter
|month=ignored (help) - ↑ Weits J, Sprenger HG, Ilic P, van Klingeren B, Elema JD, Steensma JT (1991). "[Mycobacterium avium disease in AIDS patients; diagnosis and therapy]". Ned Tijdschr Geneeskd (in Dutch; Flemish). 135 (52): 2485–9. PMID 1758516. Unknown parameter
|month=ignored (help) - ↑ Dispenzieri A, Kyle RA, Lacy MQ; et al. (2003). "POEMS syndrome: definitions and long-term outcome". Blood. 101 (7): 2496–506. doi:10.1182/blood-2002-07-2299. PMID 12456500. Unknown parameter
|month=ignored (help) - ↑ Gostiljac DM, Dordević PB, Maric-zivković J, Canović F (2005). "[Sarcoidosis localized in endocrine glands]". Med. Pregl. 58 Suppl 1: 25–9. PMID 16526262.
- ↑ 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) - ↑ Doppman JL, Gill JR, Nienhuis AW, Earll JM, Long JA (1982). "CT findings in Addison's disease". J Comput Assist Tomogr. 6 (4): 757–61. PMID 7119194. Unknown parameter
|month=ignored (help)