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Adrenal gland and paraganglia

Adrenal insufficiency

Autoimmune adrenalitis


Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 6 February 2013, last major update February 2005
Copyright: (c) 2002-2013, PathologyOutlines.com, Inc.

General
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● 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
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● 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
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● Small adrenal glands with replacement by hyalinized fibrous tissue

Micro description
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● 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
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● 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

End of Adrenal gland and paraganglia > Adrenal insufficiency > Autoimmune adrenalitis


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