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Adrenal involvement in the antiphospholipid syndrome

Department of Autoimmune Diseases, Institut Clínic d’Infeccions i Immunologia, Institut d’Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, Barcelona, Catalonia, Spain

R Cervera

Department of Autoimmune Diseases, Institut Clínic d’Infeccions i Immunologia, Institut d’Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, Barcelona, Catalonia, Spain, rcervera{at}clinic.ub.es

J Font

Department of Autoimmune Diseases, Institut Clínic d’Infeccions i Immunologia, Institut d’Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, Barcelona, Catalonia, Spain

R A Asherson

Rheumatic Diseases Unit, Department of Medicine, University of Cape Town School of Medicine, Cape Town, South Africa

Although Addison disease and hypoadrenalismare rare in patients with systemic lupus erythematosus (SLE), early reports of the associationsuggested the presence of antiphospholipidantibodies (aPL) in these patients. Data from literature reveal that adrenal failure was present in between 10 and 26% of patients with catastrophic APS and that one-third of patients presented with adrenal involvement during the course of catastrophic APS. Adrenal involvement may be the first clinical manifestation of this syndrome, whereas a few patients may have a history of Addison’s disease in the past. The pathological mechanisms involved in the production of adrenal insufficiency in APS are still not clearly understood, but the hypercoagulable state in these patients supports the concept that adrenal haemorrhagic infarction may possibly be related to adrenal vein thrombosis. In the present article, we review the pathogenic mechanisms and main clinical, laboratory and treatment features of patients suffering adrenal involvement with aPL to support the idea that APS leads to the development of adrenal insufficiency.

Key Words: Addison’s disease • adrenal insufficiency • antiphospholipid syndrome • hypoadrenalism

Lupus, Vol. 12, No. 7, 569-572 (2003)
DOI: 10.1191/0961203303lu404oa


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