Adrenal gland & paraganglia
Adrenal insufficiency
Primary and secondary


Topic Completed: 1 February 2005

Minor changes: 2 March 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Primary acute adrenal insufficiency

Nat Pernick, M.D.
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Cite this page: Pernick N. Adrenal insufficiency: primary and secondary. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalprimaryadrenalinsuff.html. Accessed May 26th, 2020.
Definition / general
Primary adrenal insufficiency:
  • Often insidious in onset, patients may present in shock due to increased stress
  • Patients usually live normal lives after diagnosis (depending on cause); may be at higher risk for heart failure, hypertension or osteopenia
  • Patients with chronic adrenal insufficiency (primary or secondary) and acute stress require immediate increase in steroids
Etiology
Primary adrenal insufficiency:
  • Rapid withdrawal of exogenous steroids (i.e. no taper) or failure to increase steroids with acute stress
  • Massive adrenal hemorrhage destroying adrenal cortex due to anticoagulation, coagulopathy and newborns with physiologic deficiencies in prothrombin time
  • Hypotension / shock that causes mild or massive corticomedullary necrosis, including Waterhouse-Friderichsen syndrome
  • Infections that destroy substantial adrenal cortical tissue
  • Amyloidosis
    • Rarely causes cortical hypofunction, only if extensive bilateral involvement
    • Usually associated with systemic amyloidosis-AA type
    • 68% of consecutive autopsies had adrenal amyloid deposits, often multinodular and probably due to aging
    • Typically affects zona fasciculata and reticularis
    • Acellular salmon-pink amorphous material is present between cortical cells, which ultimately become atrophic
    • Amyloidosis-AL type is typically deposited intravascularly
  • Drugs
    • Aminoglutethimide: inhibits enzyme converting cholesterol to pregnenolone, causes decrease in cortisol and aldosterone
    • Metapyrone: inhibits 11 beta hydroxylase, which inhibits cortisol and aldosterone synthesis
    • Mitotane: cytotoxic to zona fasciculata and reticularis, produces medical adrenalectomy; produces atrophic adrenal glands with fibrosis and residual islands of cortical cells
  • Radiation
    • May cause fibrosis, although cortex is relatively radioresistant
    • High doses (> 5000 roentgens) to abdomen, pelvis or lumbar region may cause hyaline fibrosis of reticularis and reduction of fasciculata, although does not necessarily affect cortical function
  • Autoimmune disorders (autoimmune adrenalitis or polyglandular autoimmune syndromes)
Treatment
Primary adrenal insufficiency:
  • Glucocorticoids, mineralocorticoids and IV fluids
  • In chronic patients, must give steroid boost during infections, prior to surgery or during pregnancy
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