抗磷脂抗体综合征致急性原发性肾上腺皮质功能不全1例并文献复习

Acute adrenal insufficiency caused by antiphospholipid antibody syndrome: A case report and literature review

目的探讨抗磷脂抗体综合征导致急性原发性肾上腺皮质功能不全的诊断和鉴别诊断思路。方法回顾性分析2016年5月北京协和医院内分泌科收治的1例抗磷脂抗体综合征导致双侧肾上腺出血梗死,进而出现急性原发性肾上腺皮质功能不全患者的病例资料,并复习相关文献。结果患者以腹痛起病,逐渐出现乏力、恶心、呕吐、皮肤变黑,实验室检查结果提示低钠血症,促肾上腺皮质激素升高,血皮质醇降低,糖皮质激素替代治疗后症状好转,原发性肾上腺皮质功能不全诊断明确。结合抗磷脂抗体及自身抗体阳性,双侧肾上腺出血梗死,多发动静脉血栓形成以及多系统受累表现,考虑抗磷脂抗体综合征明确,继发于系统性红斑狼疮可能性大。经过激素、免疫抑制剂以及抗凝、抗血小板治疗后,患者病情明显好转。结论对于肾上腺皮质功能不全同时伴有多系统、凝血异常以及动静脉血栓形成的患者需考虑抗磷脂抗体综合征导致肾上腺出血梗死这一罕见病因。该病的治疗需要多个科室协同合作,综合治疗,方能取得较好的预后。

Objective To investigate the diagnosis and differential diagnosis of acute primary adrenal insufficiency caused by antiphospholipid antibody syndrome. Method We retrospectively analyzed a case of anti-phospholipid antibody syndrome leading to bilateral adrenal hemorrhagic infarction, and then acute primary adrenal insufficiency patients in the endocrinology department of Peking Union Medical College Hospital in 2016, and reviewed related literature. Result A 45-year-old man complained about abdominal pain with fatigue, nausea, vomiting, and gradually darkening of the skin. Laboratory tests revealed hyponatremia, elevated adrenocorticotropic hormone, and decreased blood cortisol, as well as positive anti-phospholipid antibodies and autoantibodies. A CT imaging revealed bilateral adrenal hematoma and spleen infarction, and MRI imaging manifested with multi-cerebral infarction. His symptoms improved after glucocorticoid replacement therapy and after high dose of glucocorticoid therapy, immunosuppressive agents, and anticoagulant antiplatelet therapy, the patient’s condition improved significantly. Conclusion The rare etiology of adrenal infarction caused by anti-phospholipid antibody syndrome should be considered when patients with adrenal insufficiency also have positive anti-phospholipid antibodies, coagulopathy, and arteriovenous. The successful management of these cases requires timely and comprehensive treatment.