title: Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in the antiphospholipid syndrome: long-term outcome of 16 patients.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
authors.affiliation: Service de Médecine Interne-Endocrinologie, Centre Hospitalier Universitaire Ambroise Paré, Université Libre de Bruxelles, B-7000 Mons, Belgium.
authors.forename: Isolde
authors.initials: I
authors.lastname: Ramon
authors.forename: Alexis
authors.initials: A
authors.lastname: Mathian
authors.forename: Anne
authors.initials: A
authors.lastname: Bachelot
authors.forename: Baptiste
authors.initials: B
authors.lastname: Hervier
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Du
authors.initials: D
authors.lastname: Boutin-Le Thi Huong
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Rafik
authors.initials: R
authors.lastname: Karmali
authors.forename: Brigitte
authors.initials: B
authors.lastname: Velkeniers
authors.forename: Philippe
authors.initials: P
authors.lastname: Touraine
authors.forename: Christiane
authors.initials: C
authors.lastname: Coussieu
authors.forename: Abdelhai
authors.initials: A
authors.lastname: Bennani
authors.forename: Raphaele
authors.initials: R
authors.lastname: Renard-Penna
authors.forename: Philippe A
authors.initials: PA
authors.lastname: Grenier
authors.forename: Denis
authors.initials: D
authors.lastname: Wahl
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000224:Addison Disease / Q000209:etiology*; D000293:Adolescent; D000307:Adrenal Gland Diseases / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D000311:Adrenal Glands / Q000098:blood supply* / Q000473:pathology / Q000503:physiopathology; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications*; D002648:Child; D005260:Female; D006470:Hemorrhage / Q000150:complications*; D006801:Humans; D007238:Infarction / Q000150:complications*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies