Endocrine Abstracts

Introduction: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder known for recurrent arterial and venous thrombotic events. It may involve multiple organ systems, but its impact on the endocrine system is rare. However, the adrenal glands are one of the most susceptible endocrine organs to APS, due to thrombosis, leading to primary adrenal insufficiency (PAI). Most cases are diagnosed in patients with known history of APS.

Case Report: We report the case of 56-year-old male with history of hypertension and dyslipidemia. He was admitted to the Internal Medicine Department with a 2-week history of fatigue, anorexia, nausea, weakness and weight loss (20% in 3 months). Upon admission, he had a low blood pressure (100/54 mmHg). Blood tests revealed anemia (hemoglobin 9.9 g/dl, NR: 12.0-15.0 g/dl), thrombocytopenia (platelets 94 000×10⁶/l, NR: 150-400000×10⁶/l), elevated activated partial thromboplastin time (76.4 s, NR: 28-40 s) and hyperkaliemia (potassium 7.11 mmol/l, NR: 3.5-5.2 mmol/l). A thoracic computed tomography (CT) scan was performed and revealed a spiculated nodular lesion with 13x8mm, located in the superior left pulmonary lobe. A subsequent positron emission tomography (PET-FDG) scan was done to rule out malignancy but showed, instead, intense uptake in both adrenal glands, with heterogeneity and areas of necrosis, especially in the right adrenal gland. While in the hospital, the patient developed vomiting and postural dizziness. The endocrinology department was consulted, and hormonal assessments revealed a serum cortisol of 1.9 μg/dl (NR: 6.2-19.4 μg/dl) and adrenocorticotropic hormone (ACTH) of 626.0 pg/ml (NR: 7.2-63.3 pg/m). He was started on intravenous hydrocortisone (200 mg/day), with marked improvement on his blood pressure profile, well-being and appetite, as well as remission of nausea and vomiting. Additional tests showed negative 21-hydroxilase antibodies, along with a normal screening for infectious disease (including tuberculosis and human immunodeficiency virus). The remaining etiological investigation revealed positive lupus anticoagulant antibody, antinuclear antibody, anti-double stranded DNA antibody, and nucleosome antibody. Based on both clinical (hemorrhage/infarction of the adrenal glands) and laboratory criteria (positive antiphospholipid antibody), he was diagnosed with APS. Upon discharge, the patient was prescribed hydrocortisone (20 mg/day on divided doses), fludrocortisone 0.05 mg/day and warfarin with dose adjustments following INR (international normalized ratio) measurements. He remains asymptomatic and under regular follow-up. Subsequent MRI scans showed atrophy of the adrenal glands.

Discussion: This is a rare case of APS presenting with bilateral adrenal haemorrhage and subsequent PAI. We would like to highlight this unusual presentation of PAI, in addition to the importance of multidisciplinary teamwork in the diagnosis of such conditions.