Adrenal failure as an antiphospholipid syndrome manifestation: case-based review.

Mariana Emília Santos, Catarina Gama, Bernardo Marques, Tiago Saldanha, Joana Tremoceiro, Maria Helena Lourenço, Maria João Gonçalves, Manuela Costa, Alexandre Sepriano

Journal: ARP rheumatology 2026;4(4):305-310

PMID: 41480887

Abstract

Adrenal involvement in antiphospholipid syndrome (APS) is rare but potentially life-threatening. Early recognition reduces morbidity and mortality. We report a 56-year-old male with chronic thrombocytopenia and prolonged activated partial thromboplastin time who developed fatigue, anorexia, and weight loss. On admission, he presented with hypotension, hyperkalemia, thrombocytopenia, anemia, and elevated inflammatory markers. After hospitalization, he underwent an extensive workup study. PET-CT revealed bilateral adrenal uptake, and hormonal tests confirmed primary adrenal insufficiency (PAI). Autoimmune studies showed persistently positive lupus anticoagulant, and high-titer antinuclear and anti-double-stranded DNA antibodies, establishing systemic lupus erythematosus and APS with secondary PAI. Treatment with mineralocorticoids, hydroxychloroquine, anticoagulation, and subsequently high-dose glucocorticoids led to clinical and laboratory improvement. Review of published cases shows that, although APS is more frequent in women, adrenal involvement occurs predominantly in men and usually presents acutely with abdominal pain, hypotension, and electrolyte imbalance. Our patient's atypical presentation delayed diagnosis. Awareness of such variable presentation is crucial, as early recognition and treatment significantly improve outcomes. Clinicians should consider APS in new-onset adrenal failure and systematically evaluate adrenal involvement in APS patients with compatible symptoms.

Address: Unidade Local de Saúde de Lisboa Ocidental - Hospital de Egas Moniz, Lisbon, Portugal.; Unidade Local de Saúde de Lisboa Ocidental - Hospital Egas Moniz, Lisbon, Portugal.; Unidade Local de Saúde de Lisboa Ocidental - Hospital Egas Moniz.; Unidade Local de Saúde de Lisboa Ocidental, Lisbon, Portugal.; Unidade Local de Saúde de Entre Douro e Vouga, Santa Maria da Feira, Portugal.
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