title: Adrenal involvement in the antiphospholipid syndrome: clinical and immunologic characteristics of 86 patients.
abstract: To describe the clinical and immunologic characteristics of patients with adrenal involvement and antiphospholipid syndrome (APS), we conducted a computer-assisted (PubMed) search of the literature to identify all cases of primary adrenal insufficiency associated with antiphospholipid antibodies published in English, French, and Spanish from 1983 (when APS was first defined) through March 2002. We reviewed 86 patients (80 from the literature plus 6 from our cohort); 55% were male, and the mean age at presentation was 43 +/- 16 years. Sixty-one (71%) patients had primary APS, and 14 (16%) had systemic lupus erythematosus. In 31 (36%) patients, adrenal insufficiency was the first clinical manifestation of APS. Abdominal pain was present in 55% of patients, followed by hypotension (54%), fever (40%), nausea or vomiting (31%), weakness or fatigue (31%), and lethargy or altered mental status (19%). The main finding in imaging techniques was compatible with adrenal hemorrhage (59%) and in histopathologic study was a hemorrhagic infarction with vessel thrombosis (55%). Lupus anticoagulant was detected in 97% of patients and the anticardiolipin antibodies titer was positive in 93% of patients. Most patients (95%) were positive for the IgG isotype of anticardiolipin antibodies, whereas 40% were positive for the IgM isotype. Baseline cortisol levels were decreased in 98% of patients, ACTH hormone levels were increased in 96% of patients, and the cosyntropin stimulation test was positive in 100% of patients tested. Steroid replacement therapy was the most frequent treatment (84%), followed by anticoagulation (52%) and aspirin (6%). Thirty-two of 35 (91%) patients with prolonged anticoagulant therapy were in good health with a mean follow-up of 25 months, whereas 25 of the 69 (36%) patients with outcome data available had died. The results of the present review stress the clinical importance of systematic screening for lupus anticoagulant and anticardiolipin antibodies in all cases of adrenal hemorrhage or infarction. An initial screening for hypoadrenalism is mandatory in any antiphospholipid antibody-positive patient who complains of abdominal pain and undue weakness or asthenia.
authors.affiliation: Department of Autoimmune Diseases, Institut Clínic d'Infeccions i Immunologia, Institut d'Investigacions Biomèdiques August Pi Sunyer, Hospital Clínic, Barcelona, Catalonia, Spain.
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Eugénia
authors.initials: E
authors.lastname: Santos
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Gloria
authors.initials: G
authors.lastname: de la Red
authors.forename: Víctor
authors.initials: V
authors.lastname: Gil
authors.forename: Josep
authors.initials: J
authors.lastname: Font
authors.forename: Robert
authors.initials: R
authors.lastname: Couch
authors.forename: Miguel
authors.initials: M
authors.lastname: Ingelmo
authors.forename: Ronald A
authors.initials: RA
authors.lastname: Asherson
mesh_terms: D015746:Abdominal Pain / Q000209:etiology / Q000628:therapy; D000293:Adolescent; D000311:Adrenal Glands / Q000000981:diagnostic imaging / Q000473:pathology; D000309:Adrenal Insufficiency / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D000328:Adult; D000368:Aged; D017153:Antibodies, Anticardiolipin / Q000097:blood; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000276:immunology / Q000628:therapy; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007132:Immunoglobulin Isotypes / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D017153:Antibodies, Anticardiolipin; D000925:Anticoagulants; D005938:Glucocorticoids; D007132:Immunoglobulin Isotypes; D007166:Immunosuppressive Agents; D016682:Lupus Coagulation Inhibitor