Antiphospholipid syndrome with adrenal insufficiency

Case contributed by Ammar Ashraf
Diagnosis almost certain

Presentation

Known case of antiphospholipid syndrome with adrenal insufficiency.

Patient Data

Age: 45 years
Gender: Female

Average-size adrenal glands. No focal solid or cystic abnormality, calcifications or hemorrhage is appreciable in either adrenal gland. Both adrenal glands show poor enhancement on the post-contrast images. Renal arteries & veins are patent and well opacified. An incidental note is made of the right hepatic artery arising from the SMA and coursing between the portal vein and IVC (aberrant replaced right hepatic artery). Superior endplate compression fracture of the first lumbar vertebra and mild wedging of the 10th dorsal vertebra, related to a history of previous trauma.

Case Discussion

  • Considering the history of antiphospholipid syndrome, CT scan findings of poorly enhancing adrenal glands are suggestive of adrenal insufficiency.

  • Adrenal glands are the most frequently affected endocrine glands in antiphospholipid syndrome (APS) 1. Adrenal insufficiency (AI) or Addison disease (AD) is now a well-recognized and the commonest endocrine manifestation of the APS and routine evaluation of the adrenal functions is recommended in APS 1,2. Approximately, in one-third of the cases, AI may be the first clinical manifestation of APS 1-4. Adrenal vein thrombosis leading to spontaneous hemorrhagic infarction or hemorrhagic necrosis is most likely the cause of AI in APS 1,3. The unique vascular supply of the adrenal glands (rich arterial supply with limited venous drainage) may be an important underlying predisposing factor for such thrombosis 2,3.

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