Shahrrava Anahita, Moinuddin Sunnan, Boddu Prajwal, Shah Rohan
Department of Internal Medicine, Advocate Illinois Masonic Medical Center, 836 West Wellington Avenue, Chicago, IL 60657, USA.
Department of Radiology, Advocate Illinois Masonic Medical Center, 836 West Wellington Avenue, Chicago, IL 60657, USA.
Case Rep Endocrinol. 2016;2016:2017571. doi: 10.1155/2016/2017571. Epub 2016 Jun 5.
Glucocorticoid remediable aldosteronism (GRA) is rare familial form of primary aldosteronism characterized by a normalization of hypertension with the administration of glucocorticoids. We present a case of GRA and thoracoabdominal aneurysm complicated by multiple aortic dissections requiring complex surgical and endovascular repairs. Registry studies have shown a high rate of intracranial aneurysms in GRA patients with high case fatality rates. The association of thoracoabdominal aneurysms with GRA has not been described, thus far, in literature. Studies have shown that high tissue aldosterone levels concomitant with salt intake have a significant role in the pathogenesis of aneurysms and this may explain the formation of aneurysms in the intracranial vasculature and aorta. The association of GRA with thoracic aortic aneurysms needs to be further studied to develop screening recommendations for early identification and optimal treatment. Also, the early use of mineralocorticoid antagonists may have a significant preventive and attenuating effect in aneurysm formation, an association which needs to be confirmed in future studies.
糖皮质激素可治性醛固酮增多症(GRA)是一种罕见的原发性醛固酮增多症的家族形式,其特征是使用糖皮质激素后高血压得以恢复正常。我们报告一例GRA合并胸腹主动脉瘤并伴有多处主动脉夹层,需要进行复杂的外科手术和血管腔内修复。登记研究表明,GRA患者颅内动脉瘤的发生率很高,病死率也很高。迄今为止,文献中尚未描述胸腹主动脉瘤与GRA的关联。研究表明,高组织醛固酮水平与盐摄入相伴在动脉瘤的发病机制中起重要作用,这可能解释了颅内血管和主动脉中动脉瘤的形成。GRA与胸主动脉瘤的关联需要进一步研究,以制定早期识别和最佳治疗的筛查建议。此外,早期使用盐皮质激素拮抗剂可能对动脉瘤形成具有显著的预防和减轻作用,这种关联需要在未来的研究中得到证实。