Endocrine Abstracts

Introduction: Autoimmune adrenal injury is the most common cause of primary adrenal failure (PAF) (70–90%). Antiphospholipid syndrome (APS) that characterized by thrombosis and abortus may be a reason of PAF. In this presentation we reported a case of primary adrenal failure due to primary antiphospholipid syndrome.

Case: A 34-year-old boy was brought to emergency service due to loss of consciousness. In physical examination patients was confused, blood pressure was 70/50 mmHg, he had widespread hyperpigmentation on his skin and oral mucosa and there was scars in both legs. In Laboratory examination were as follow; blood glucose 37 mg/dl, creatinine 1 mg/dl, sodium 135 mmol/l, potassium 6 mmol/l, thrombocyte count 83 K/ul (140–440), cortisol 1.91 μg/dl and ACTH550 pg/ml. Acute adrenal crisis treatment started and patient hospitalized to evaluate the causes of PAF. Patient was evaluated for possible causes of PAF in terms of HIV, syphilis, disseminated fungal infections and tuberculosis. He had no history for drugs that cause adrenal insufficiency. Bilateral low extremity doppler ultrasound performed due to scars in his legs. Doppler ultrasound revealed thrombosis in bilateral femoral vein, right deep femoral vein, superficial femoral vein, popliteal vein and right vena saphena magna. For thrombosis etiology blood sample were taken for proteinC, proteinS, aPTT and PT%, results were as follow respectively; 85.2% (70–140), 82.4% (60–130), 77.7 sec (20–35), 15 sec (11–16). Antiphospholipid syndrome was suspected due to increase aPTT levels and thrombosis. Laboratory tests for antiphospholipid antibodies revealed increased anticardiolipin IgG, anticardiolipin IgM and anti β-2 glycoprotein IgG. After 12 weeks repeated test for antiphospholipid antibodies were also high. Due to revised sapporo criteria patient was diagnosed as antiphospholipid syndrome.

Discussion: Antiphospholipid syndrom is characterized with antiphospholipid antibodies. Antiphospholipid antibodies affect coagulation pathway and cause clinical manifestations. APS may affects many organs and one of them is adrenal gland. Adrenal failure in APS is probably related to spontaneous hemorrhagic infarct and adrenal vein thrombosis. Antigens such as lysobiphosphatidic acid in zona fasciculata is target for antiphospholipid antibodies and this is a possible mechanism for adrenal failure in APS. We should keep in mind APS for a possible cause of adrenal insufficiency.