Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in the antiphospholipid syndrome: long-term outcome of 16 patients

J Clin Endocrinol Metab. 2013 Aug;98(8):3179-89. doi: 10.1210/jc.2012-4300. Epub 2013 Jun 19.


Context: Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction is a rare and life-threatening manifestation of the antiphospholipid syndrome (APLS). Data on the long-term outcome are scarce.

Objective: The aims of the present study were to analyze the long-term outcome related to APLS per se and to characterize the course of adrenal involvement.

Design: We conducted a retrospective study of patients with bilateral adrenal hemorrhage-adrenal infarction secondary to APLS seen in the Department of Internal Medicine of Pitié-Salpêtrière Hospital in Paris (France) between January 1990 and July 2010.

Results: Three patients died during the acute phase related to APLS manifestations. Sixteen patients (7 males; 9 females) were followed up during a median period of 3.5 years (range 0.3-28.1 years). Three episodes of recurrent thrombosis were noted. One patient died from cerebral hemorrhage 3 months after the onset of adrenal insufficiency. Repeated Synacthen tests showed complete absence of response in 8 of the 10 patients assessed; cortisol and aldosterone increased appropriately in one patient and to some extent in another one. Dehydroepiandrosterone levels and 24-hour urinary epinephrine levels remained abnormally low in all evaluated patients. Adrenal imaging performed more than 1 year after the initial event revealed completely atrophic glands in 9 of 11 patients.

Conclusions: This particular subset of APLS patients who survive the acute phase has a rather favorable long-term outcome. Although adrenal dysfunction is generally irreversible, adrenocortical function may, at least partially, recover in rare cases. In this view, measurement of early morning cortisol during follow-up is indicated to detect these patients.

MeSH terms

  • Addison Disease / etiology*
  • Adolescent
  • Adrenal Gland Diseases / complications*
  • Adrenal Gland Diseases / pathology
  • Adrenal Gland Diseases / physiopathology
  • Adrenal Glands / blood supply*
  • Adrenal Glands / pathology
  • Adrenal Glands / physiopathology
  • Adult
  • Antiphospholipid Syndrome / complications*
  • Child
  • Female
  • Hemorrhage / complications*
  • Humans
  • Infarction / complications*
  • Male
  • Middle Aged
  • Retrospective Studies