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Adrenal involvement in the antiphospholipid syndrome
G Espinosa1, R Cervera, J Font
1Department of Autoimmune Diseases, Institut Clinic d'Infeccions i Immunologia, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clinic, Barcelona, Catalonia, Spain.
Lupus
|August 2, 2003
Summary
Antiphospholipid syndrome (APS) can cause adrenal insufficiency, a rare condition in systemic lupus erythematosus (SLE) patients. This review explores the link between APS, antiphospholipid antibodies (aPL), and adrenal failure, highlighting potential mechanisms like adrenal vein thrombosis.
Area of Science:
- Endocrinology
- Rheumatology
- Pathology
Background:
- Addison disease and hypoadrenalism are rare in systemic lupus erythematosus (SLE) patients.
- Early reports suggested a link between SLE, antiphospholipid antibodies (aPL), and adrenal issues.
- Adrenal failure occurs in 10-26% of catastrophic APS cases and can be an initial manifestation.
Purpose of the Study:
- To review pathogenic mechanisms, clinical, laboratory, and treatment features of adrenal involvement in antiphospholipid syndrome (APS).
- To support the hypothesis that APS leads to adrenal insufficiency.
- To elucidate the role of antiphospholipid antibodies (aPL) in APS-related adrenal compromise.
Main Methods:
- Literature review of pathogenic mechanisms, clinical presentations, laboratory findings, and treatment of adrenal involvement in APS.
- Analysis of existing data on the prevalence of adrenal failure in catastrophic APS.
- Synthesis of evidence linking hypercoagulable states in APS to adrenal pathology.
Main Results:
- Adrenal failure is a significant complication in catastrophic APS, affecting 10-26% of patients.
- Adrenal involvement can be the primary clinical presentation of APS.
- Adrenal vein thrombosis due to hypercoagulability is a potential mechanism for adrenal hemorrhagic infarction in APS.
Conclusions:
- Antiphospholipid syndrome (APS) is a significant cause of adrenal insufficiency.
- Antiphospholipid antibodies (aPL) play a crucial role in the pathogenesis of APS-related adrenal compromise.
- Understanding these mechanisms is vital for timely diagnosis and management of adrenal involvement in APS.