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Definition / general | Case reports | Gross description | Microscopic (histologic) description | Differential diagnosisCite this page: Pernick N. Autoimmune adrenalitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalautoimmuneadrenalitis.html. Accessed March 19th, 2024.
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