Adrenal insufficiency

Changed by Yaïr Glick, 18 Jun 2017

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Adrenal insufficiency refers to inadequate secretion of corticosteroids (glucocorticoids and mineralocorticoids).

It may occur from partial or complete destruction of the adrenal gland cortex, in which case it is termed "primaryprimary adrenal insufficiency" (also known as "Addison disease"). Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common aetiology overall.

Clinical presentation

Depends on the course of the disease:

  • acute stage: the patient presents with fever, back pain, hypotension, weakness
  • chronic stage: progressive lethargy, weakness, cutaneous pigmentation, weight loss
Laboratory data:
  • chemistry: hyponatremia, hyperkalemia, azotemia, hypercalcemia, hypoglycemia
  • adrenocorticotrophic hormone (ACTH) stimulation test: cortisol level will fail to fall 

Pathology

Primary
  • idiopathic autoimmune disorders which are the most common cause in developed countries (80% of cases) in developed countries 1
  • granulomatous disease: tuberculosis (the most common infectious cause in underdevelopeddeveloping countries) and sarcoidosis
  • neoplasms: metastases (e.g. lung, ovary, kidney, melanoma), lymphoma, and leukaemia
  • adrenal haemorrhage: shock, sepsis (Waterhouse-Friderichsen syndrome), coagulation disorders, antiphospholipid syndrome
  • systemic fungal infection: histoplasmosis (most common infection in the southeastern and south central United States 1)
  • adrenoleukodystrophy
Secondary
Time course

The disease course may be either acute, subacute, or chronic 2:

  • acute: occurs within a few weeks to months and is caused by bilateral adrenal haemorrhage (adrenal apoplexy) or secondary to shock and sepsis or trauma (Addisonian crisis)
  • subacute disease (adrenalitis): when the disease has been present for less than two years
  • chronic: secondary to chronic autoimmune disorder or chronic granulomatous infection (tuberculosis)
    • the adrenal glands become atrophic and calcified

Radiographic features

Adrenal insufficiency is a bilateral process that cannot be diagnosed by imaging alone. CT would be the best imaging modalitiesmodality for adrenal assessment in this adrenal insufficiency but is not necessary for diagnosis.

CT

Imaging features depend on the cause and the course of the disease either acute, subacute or chronic:

  • acute: bilateral adrenal hematomas
  • subacute (adrenalitis): enlargement of both adrenal glands, with necrotic centres and peripheral enhancing rims
  • chronic: both adrenal glands appear small and atrophic, associated with calcifications (adrenal calcification) in granulomatous adrenalitis

Treatment and prognosis

  • acute: glucocorticoid therapy, volume and electrolyte replacement 
  • chronic: glucocorticoid and mineralocorticoid replacement

Differential diagnosis

  • -<p><strong>Adrenal insufficiency </strong>refers to inadequate secretion of corticosteroids and mineralocorticoids.</p><p>It may occur from partial or complete destruction of the <a href="/articles/adrenal-gland">adrenal gland</a> cortex, in which case it is termed "primary adrenal insufficiency" (also known as "<strong>Addison disease</strong>"). Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common aetiology overall.</p><h4>Clinical presentation</h4><p>Depends on the course of the disease:</p><ul>
  • -<li>acute stage: the patient presents with fever, back pain, hypotension, weakness</li>
  • -<li>chronic stage: progressive lethargy, weakness, cutaneous pigmentation, weight loss</li>
  • -</ul><p>Laboratory data:</p><ul>
  • +<p><strong>Adrenal insufficiency </strong>refers to inadequate secretion of corticosteroids (glucocorticoids and mineralocorticoids).</p><p>It may occur from partial or complete destruction of the <a href="/articles/adrenal-gland">adrenal</a> cortex, in which case it is termed <strong>primary adrenal insufficiency</strong> (also known as <strong>Addison disease</strong>). Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common aetiology overall.</p><h4>Clinical presentation</h4><p>Depends on the course of the disease:</p><ul>
  • +<li>
  • +<strong>acute stage:</strong> the patient presents with fever, back pain, hypotension, weakness</li>
  • +<li>
  • +<strong>chronic stage</strong>: progressive lethargy, weakness, cutaneous pigmentation, weight loss</li>
  • +</ul><h5>Laboratory data</h5><ul>
  • -<li>idiopathic autoimmune disorders which are the most common cause (80% of cases) in developed countries <sup>1</sup>
  • +<li>idiopathic autoimmune disorders are the most common cause in developed countries (80% of cases) <sup>1</sup>
  • -<li>granulomatous disease: <a href="/articles/tuberculosis">tuberculosis</a> (the most common infectious cause in underdeveloped countries) and <a href="/articles/sarcoidosis-1">sarcoidosis</a>
  • +<li>granulomatous disease: <a href="/articles/tuberculosis">tuberculosis</a> (the most common infectious cause in developing countries) and <a href="/articles/sarcoidosis-1">sarcoidosis</a>
  • -<li>systemic fungal infection: histoplasmosis (most common infection in the southeastern and south central United States <sup>1</sup>)</li>
  • +<li>systemic fungal infection: histoplasmosis (most common in the southeastern and south central United States <sup>1</sup>)</li>
  • -<li>hypothalamic or pituitary lesions</li>
  • +<li>
  • +<a title="Hypothalamic lesions" href="/articles/hypothalamic-lesions">hypothalamic</a> or <a title="Approach to pituitary masses" href="/articles/pituitary-mri-an-approach-1">pituitary lesions</a>
  • +</li>
  • -</ul><h4>Radiographic features</h4><p>Adrenal insufficiency is bilateral process that cannot be diagnosed by imaging alone. CT would be the best imaging modalities for adrenal assessment in this adrenal insufficiency but is not necessary for diagnosis.</p><h5>CT</h5><p>Imaging features depend on the cause and the course of the disease either acute, subacute or chronic:</p><ul>
  • +</ul><h4>Radiographic features</h4><p>Adrenal insufficiency is a bilateral process that cannot be diagnosed by imaging alone. CT would be the best imaging modality for adrenal assessment but is not necessary for diagnosis.</p><h5>CT</h5><p>Imaging features depend on the cause and the course of the disease either acute, subacute or chronic:</p><ul>
  • -<strong>chronic</strong>: both adrenal glands appear small and atrophic associated with calcifications (<a href="/articles/adrenal-calcification">adrenal calcification</a>) in granulomatous adrenalitis </li>
  • +<strong>chronic</strong>: both adrenal glands appear small and atrophic, associated with calcifications (<a href="/articles/adrenal-calcification">adrenal calcification</a>) in granulomatous adrenalitis</li>

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