Tauchmanovà L, Rossi R, Coppola A, Luciano A, Del Viscovo L, Soriente L, De Bellis A, Di Minno G, Lombardi G
Dipartimento di Endocrinologia ed Oncologia Molecolare e Clinica, Università degli Studi Federico II, Naples, Italy.
Eur J Endocrinol. 1998 Dec;139(6):641-5. doi: 10.1530/eje.0.1390641.
The antiphospholipid syndrome is characterized by clinical evidence of arterial or venous thrombosis, thrombocytopaenia, recurrent fetal loss and repeated positivity of antiphospholipid autoantibodies. The association of antiphospholipid syndrome with the development of adrenal failure has been reported in more than 40 patients in the last 20 years, mostly due to bilateral cortical haemorrhage or thrombosis of adrenal vessels. The presence of antibodies against adrenal cortex was never documented in these patients. Here we report a case of recurrent thrombophlebitis, acute adrenal failure, and chronic hepatitis occurring in a young man found to have antiphospholipid antibodies and lupus anticoagulant. Autoantibodies against adrenal cortex were detected and abdominal ultrasonography showed morphologically normal adrenals. Mild thrombocytopaenia, Coomb's positive anaemia, increase in alanine- and aspartate-aminotransferases and increase in urinary protein excretion were found. Autoantibodies against liver/kidney microsomes were positive and liver biopsy was compatible with autoimmune hepatitis. The patient was treated with cortisone acetate, fludrocortisone and warfarin. Dilated cardiomyopathy was revealed one year later and coronarography did not document any occlusive coronary disease. Three years later, titres of autoantibodies, including those directed towards the adrenal cortex, were increased and others, previously absent, were detected. Nevertheless, the patient's clinical conditions seemed unchanged. At this time, an abdominal CT scan showed adrenal dysmorphisms with bilateral annular calcifications and central hypodensities suggesting previous bilateral adrenal haematomas. The hypercoagulable state that occurs in antiphospholipid syndrome can induce a localized inflammatory response generated by tissue injury, with a consequent release of intracellular antigens and antibodies production. Consequently, tissue-specific autoantibodies positivity may persist until the cells involved in antigen production are completely destroyed.
抗磷脂综合征的特征为存在动脉或静脉血栓形成、血小板减少、反复流产以及抗磷脂自身抗体反复阳性的临床证据。在过去20年里，已有40多名患者报告了抗磷脂综合征与肾上腺功能衰竭发生之间的关联，主要原因是双侧肾上腺皮质出血或肾上腺血管血栓形成。这些患者中从未记录到抗肾上腺皮质抗体的存在。在此，我们报告一例发生在一名年轻男性身上的复发性血栓性静脉炎、急性肾上腺功能衰竭和慢性肝炎病例，该患者被发现存在抗磷脂抗体和狼疮抗凝物。检测到了抗肾上腺皮质自身抗体，腹部超声显示肾上腺形态正常。发现有轻度血小板减少、库姆斯试验阳性贫血、丙氨酸转氨酶和天冬氨酸转氨酶升高以及尿蛋白排泄增加。抗肝肾微粒体自身抗体呈阳性，肝活检结果符合自身免疫性肝炎。该患者接受了醋酸可的松、氟氢可的松和华法林治疗。一年后发现有扩张型心肌病，冠状动脉造影未发现任何闭塞性冠状动脉疾病。三年后，包括针对肾上腺皮质的自身抗体在内的自身抗体滴度升高，并且检测到了其他之前不存在的自身抗体。然而，患者的临床状况似乎没有变化。此时，腹部CT扫描显示肾上腺形态异常，双侧有环形钙化和中央低密度影，提示既往有双侧肾上腺血肿。抗磷脂综合征中出现的高凝状态可诱导由组织损伤产生的局部炎症反应，从而导致细胞内抗原释放和抗体产生。因此，组织特异性自身抗体阳性可能会持续存在，直到参与抗原产生的细胞被完全破坏。
