Adrenal gland and paraganglia
Adrenal insufficiency
Autoimmune adrenalitis


Topic Completed: 01 February 2005

Revised: 20 December 2018

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PubMed Search: Autoimmune adrenalitis
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Cite this page: Pernick N. Autoimmune adrenalitis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalautoimmuneadrenalitis.html. Accessed January 23rd, 2019.
Definition / general
  • Also called idiopathic primary adrenal insufficiency
  • No clinical findings until 90% of adrenal cortex is destroyed
  • Causes 70-90% of cases of adrenal insufficiency; usually women ages 20-50, sporadic or familial
  • Up to 75% have autoantibodies against adrenal cortical zones not present in normal patients; antibodies appear months to years before onset of adrenal insufficiency
  • 50% of autoimmune cases have circulating autoantibodies to 21 hydroxylase and 17 alpha-hydroxylase enzymes
  • 60% are associated with Hashimoto thyroiditis, pernicious anemia, type 1 diabetes or idiopathic hypoparathyroidism (although patients with these common disorders only rarely develop adrenal insufficiency)
  • Associated with HLA-B8, DR3 and DR4
  • 45% with circulating autoantibodies, but without symptoms develop impaired adrenocortical function within 2 1/2 years
Case reports
  • 24 year old woman with death due to pituitary and adrenal insufficiency, with heavy lymphocytic infiltration of adenohypophysis, thyroid, adrenals and diffuse retroperitoneal fibrosis with perivascular lymphocytic infiltrates, 2 years after delivery of normal infant (Arch Pathol Lab Med 1985;109:230)
  • Due to intravascular B cell lymphoma (Hum Pathol 1996;27:209)
Gross description
  • Small adrenal glands with replacement by hyalinized fibrous tissue
Microscopic (histologic) description
  • Fibrotic capsule
  • Lymphocytes, histiocytes and plasma cells in all cortical layers
  • Rare or small islands of remaining cortical cells with eosinophilic cytoplasm and lipid depletion
  • Medulla is unchanged
Differential diagnosis
  • Carney complex: lymphocytes and nodules of enlarged zona reticularis-type cells, no glandular atrophy
  • Chronic glucocorticoid therapy: atrophic adrenal glands but no inflammation, no adrenal insufficiency except in times of crisis
  • Myelipomatous change: fat cells, lymphocytes and bone marrow elements
  • Normal adrenal cortex: focal lymphocytic aggregates, but cortical cells present and no symptoms of adrenal insufficiency