2型自身免疫性多腺综合征患者低容量渗出性缩窄性心包炎所致的心包填塞

William C Palmer, Andrew Kurklinsky, Gary Lane, Kamonpun Ussavarungsi, Joseph L Blackshear
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引用次数: 9

摘要

II型自身免疫性多腺综合征(APS)是一种较为常见的内分泌疾病,包括原发性肾上腺功能不全合并1型糖尿病和/或自身免疫性原发性甲状腺功能减退。自身免疫性浆液炎是一种相关疾病,可表现为症状性心包积液。我们提出一个54岁的男性APS谁发展心包炎导致心包填塞与亚急性局部积液。紧急心包穿刺后心包内压降至0,中心静脉压升高,符合急性渗出性缩窄性心包炎。计算机断层造影证实心包造影增强。患者在长期肌力支持和糖皮质激素治疗后恢复。16天后积液再次积聚,需要再次心包穿刺。积液性缩窄性心包炎是一种罕见的心包综合征,其特征是心包炎症和心包填塞同时发生。虽然没有很好的血流动力学结果,但已有报道的APS合并心包填塞的病例,尽管积液量小。我们报告一例因心包炎和明显低血压引起的反复心包积液的APS病例,并进行全面的临床和血流动力学评估。这些患者可能需要积极的支持,如心包穿刺术、肌力疗法和激素替代疗法。对于复发性填塞应密切随访。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome.

Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive-constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.

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