Espinosa Gerard, Santos Eugénia, Cervera Ricard, Piette Jean-Charles, de la Red Gloria, Gil Víctor, Font Josep, Couch Robert, Ingelmo Miguel, Asherson Ronald A
Department of Autoimmune Diseases, Institut Clínic d'Infeccions i Immunologia, Institut d'Investigacions Biomèdiques August Pi Sunyer, Hospital Clínic, Barcelona, Catalonia, Spain.
Medicine (Baltimore). 2003 Mar;82(2):106-18. doi: 10.1097/00005792-200303000-00005.
To describe the clinical and immunologic characteristics of patients with adrenal involvement and antiphospholipid syndrome (APS), we conducted a computer-assisted (PubMed) search of the literature to identify all cases of primary adrenal insufficiency associated with antiphospholipid antibodies published in English, French, and Spanish from 1983 (when APS was first defined) through March 2002. We reviewed 86 patients (80 from the literature plus 6 from our cohort); 55% were male, and the mean age at presentation was 43 +/- 16 years. Sixty-one (71%) patients had primary APS, and 14 (16%) had systemic lupus erythematosus. In 31 (36%) patients, adrenal insufficiency was the first clinical manifestation of APS. Abdominal pain was present in 55% of patients, followed by hypotension (54%), fever (40%), nausea or vomiting (31%), weakness or fatigue (31%), and lethargy or altered mental status (19%). The main finding in imaging techniques was compatible with adrenal hemorrhage (59%) and in histopathologic study was a hemorrhagic infarction with vessel thrombosis (55%). Lupus anticoagulant was detected in 97% of patients and the anticardiolipin antibodies titer was positive in 93% of patients. Most patients (95%) were positive for the IgG isotype of anticardiolipin antibodies, whereas 40% were positive for the IgM isotype. Baseline cortisol levels were decreased in 98% of patients, ACTH hormone levels were increased in 96% of patients, and the cosyntropin stimulation test was positive in 100% of patients tested. Steroid replacement therapy was the most frequent treatment (84%), followed by anticoagulation (52%) and aspirin (6%). Thirty-two of 35 (91%) patients with prolonged anticoagulant therapy were in good health with a mean follow-up of 25 months, whereas 25 of the 69 (36%) patients with outcome data available had died. The results of the present review stress the clinical importance of systematic screening for lupus anticoagulant and anticardiolipin antibodies in all cases of adrenal hemorrhage or infarction. An initial screening for hypoadrenalism is mandatory in any antiphospholipid antibody-positive patient who complains of abdominal pain and undue weakness or asthenia.
为描述肾上腺受累及抗磷脂综合征（APS）患者的临床和免疫学特征，我们通过计算机辅助（PubMed）检索文献，以确定1983年（APS首次被定义）至2002年3月期间以英文、法文和西班牙文发表的所有与抗磷脂抗体相关的原发性肾上腺功能不全病例。我们回顾了86例患者（80例来自文献，6例来自我们的队列）；55%为男性，就诊时的平均年龄为43±16岁。61例（71%）患者患有原发性APS，14例（16%）患有系统性红斑狼疮。在31例（36%）患者中，肾上腺功能不全是APS的首发临床表现。55%的患者有腹痛，其次是低血压（54%）、发热（40%）、恶心或呕吐（31%）、虚弱或疲劳（31%）以及嗜睡或精神状态改变（19%）。影像学检查的主要发现与肾上腺出血相符（59%），组织病理学研究的主要发现是伴有血管血栓形成的出血性梗死（55%）。97%的患者检测到狼疮抗凝物，93%的患者抗心磷脂抗体滴度呈阳性。大多数患者（95%）抗心磷脂抗体的IgG亚型呈阳性，而40%的患者IgM亚型呈阳性。98%的患者基线皮质醇水平降低，96%的患者促肾上腺皮质激素水平升高，100%接受检测的患者促肾上腺皮质激素刺激试验呈阳性。类固醇替代疗法是最常用的治疗方法（84%），其次是抗凝治疗（52%）和阿司匹林治疗（6%）。35例接受长期抗凝治疗的患者中有32例（91%）健康状况良好，平均随访25个月，而69例有结局数据的患者中有25例（36%）死亡。本综述结果强调了在所有肾上腺出血或梗死病例中系统筛查狼疮抗凝物和抗心磷脂抗体的临床重要性。对于任何主诉腹痛以及过度虚弱或乏力的抗磷脂抗体阳性患者，必须进行肾上腺功能减退的初步筛查。
