Endocrine Abstracts

Introduction: We report a case of adrenal insufficiency as the first clinical manifestation of antiphospholipid syndrome.

Case report: A 56-year-old male with no previous history of thrombosis was admitted with 3 stone weight loss and feeling generally unwell. He was hypotensive, pigmented and had a slate grey pigmentation of the nose. Chest auscultation revealed reduced breath sounds. Investigations revealed low sodium and high potassium levels with metabolic acidosis. Synacthen test revealed a flat response but adrenal antibody was negative. Chest radiograph and CT scan showed bilateral pleural effusions. CT scan of the abdomen showed bilateral adrenal masses. With fluid resuscitation, intravenous antibiotics and steroid replacement, his symptoms had resolved. Screening for pheochromocytoma was negative and biopsy of the adrenals showed necrotic tissue. Antiphospholipid antibody (IgM) was strongly positive. The patient was re-evaluated with abdominal CT scan which still showed bilaterally enlarged adrenals. Genetic analysis showed compound heterozygote for haemochromatosis. He was referred to haematologist for further follow up.

Discussion: The incidence of Addison’s disease is 4.7–6.2 per million population. Antiphospholipid syndrome is diagnosed in <0.5% of all patients with addison’s disease. Our patient was interesting as adrenal insufficiency was the first manifestation of the antiphosholipid syndrome. In addition the presence of haemochromatosis clouded the clinical picture further. Literature search had revealed that only 20 patients had presented with addisons disease as a first manifestation of antiphospholipid syndrome. Out of them 75% of them were males. Abdominal pain followed by fever and hypotension were the main symptoms. The recognition of the beta 2 glycoprotein 1 by the antiphopsholipid antibodies on the surface epithelial cells may explain the procoagulant state in antiphopholipid syndrome.

Conclusion: Antiphopholipid syndrome should be considered as a possible aetiology in patients presenting with addison’s disease where the aetiology is not obvious.