Boscaro M, Betterle C, Sonino N, Volpato M, Paoletta A, Fallo F
Division of Endocrinology, University of Padova, Italy.
J Clin Endocrinol Metab. 1994 Aug;79(2):452-5. doi: 10.1210/jcem.79.2.8045962.
Idiopathic Addison's disease occurs frequently in association with other organ-specific autoimmune diseases, and autoantibodies to adrenal cortex are markers of this condition. A variable asymptomatic period with subtle adrenal dysfunction may precede the onset of clinical manifestations. We studied the pituitary-adrenal axis by measuring plasma ACTH, cortisol, and 17 alpha-hydroxyprogesterone after ovine CRH (100 micrograms as an iv bolus) stimulation in 19 patients with organ-specific autoimmune disease and adrenal autoantibodies, in whom adrenal steroids were normal under baseline conditions and normally responsive to a standard ACTH stimulation test (250 micrograms). In all subjects, oCRH produced a normal increase in plasma ACTH. Plasma cortisol, which was normoresponsive in 11 subjects, showed little or no increase in 8 subjects. Two of these patients developed overt adrenal failure after 1 yr. The 17 alpha-hydroxyprogesterone response to oCRH, tested in 10 of 19 patients, paralleled that of plasma cortisol, excluding a steroidogenic block at the 21-hydroxylase site. Our data demonstrate the existence of a very early phase of Addison's disease in which adrenal function shows an impaired response to ovine CRH-stimulated ACTH.
特发性Addison病常与其他器官特异性自身免疫性疾病相关，肾上腺皮质自身抗体是这种疾病的标志物。在临床表现出现之前，可能存在一个肾上腺功能轻微异常的无症状期。我们通过静脉注射100微克羊促肾上腺皮质激素释放激素（oCRH）后测量血浆促肾上腺皮质激素（ACTH）、皮质醇和17α-羟孕酮，对19例患有器官特异性自身免疫性疾病且有肾上腺自身抗体的患者的垂体-肾上腺轴进行了研究，这些患者在基线条件下肾上腺类固醇正常，对标准ACTH刺激试验（250微克）反应正常。在所有受试者中，oCRH使血浆ACTH正常升高。11名受试者的血浆皮质醇反应正常，8名受试者的血浆皮质醇几乎没有增加或没有增加。其中两名患者在1年后出现明显的肾上腺功能衰竭。在19名患者中的10名患者中检测到的17α-羟孕酮对oCRH的反应与血浆皮质醇的反应相似，排除了21-羟化酶位点的类固醇生成阻滞。我们的数据表明，Addison病存在一个非常早期的阶段，在此阶段肾上腺功能对羊CRH刺激的ACTH反应受损。
