Detection of anticonductive tissue autoantibodies in a patient with chronic intestinal pseudo-obstruction and sick sinus syndrome.
Detection of anticonductive tissue autoantibodies in a patient with chronic intestinal pseudo-obstruction and sick sinus syndrome.
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DOI：
10.1097/meg.0b013e3283632dbc
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发表时间：
2013-11
影响因子：
2.1
通讯作者：
De Giorgio R
中科院分区：
文献类型：
--
作者：
Caio G;Volta U;Cerrato E;Clavenzani P;Montali N;Cogliandro R;Stanghellini V;Golzio PG;Gaita F;Farrugia G;De Giorgio R
一名26岁的病人被诊断为患有慢性假性肠梗阻与测压和组织病理学特征提示肠肌病。组织学特征为平滑肌变性，无炎症或免疫细胞。严重的肠道功能障碍需要充分的肠外营养支持。几个月后，患者出现症状性快速-缓慢心律失常发作伴晕厥。全面的诊断性检查诊断为病窦综合征，通过起搏器植入和β受体阻滞剂给药进行治疗。这导致快速-缓慢心律失常发作的部分改善。尽管如此，患者继续出现持续性室上性快速性心律失常，对β受体阻滞剂剂量增加反应不良。为了调查心脏损害的起源，对患者进行了抗传导组织自身抗体检测，结果呈阳性，从而支持心律失常可能是自身免疫起源。其他自身抗体检测均为阴性。基于这些结果，患者接受高剂量类固醇治疗，然后逐渐减量。患者对类固醇治疗有反应，未再发生晕厥和快速性心律失常。严重的肠道功能障碍保持不变。这个病例强调了严重的肠道功能障碍和心脏传导组织异常之间的联系，传导组织自身抗体可能导致心律失常。本病例中出现的严重肠道和心脏（可能是自身免疫介导的）功能障碍为进一步评估肠道和心脏异常节律之间的联系提供了基础。
A 26-year-old patient was diagnosed as suffering from chronic intestinal pseudo-obstruction with manometric and histopathologic features suggestive of a intestinal myopathy. Histology was characterized by smooth muscle degeneration without inflammatory or immune cells. The severe gut dysfunction required full parenteral nutritional support. Few months later, the patient developed symptomatic tachy-brady arrhythmia episodes with syncopes. A thorough diagnostic work-up led to a diagnosis of sick sinus syndrome which was managed by pacemaker implantation and β-blockers administration. This led to a partial improvement of tachy-brady arrhythmia episodes. Nonetheless, the patient continued to experience sustained supraventricular tachyarrhythmia runs, poorly responsive to increasing β-blocker doses. To investigate the origin of the cardiologic impairment, the patient was tested for anti-conductive tissue autoantibodies, which were positive, thus supporting a possible autoimmune origin of the dysrhythmia. Other autoantibodies tested for were negative. Based on these findings, the patient was treated with high dose steroids which were then tapered. The patient responded to the steroid treatment and did not experience further episodes of syncope and tachyarrhythmias. The severe gut dysfunction remained unchanged. This case highlights an association between severe gut dysfunction and cardiac conductive tissue abnormalities with autoantibodies to conductive tissue possibly causing the dysrhythmia. The severe gut and heart (likely autoimmune-mediated) dysfunction presented in this case provide a basis to assess further a link between intestinal and cardiac abnormal rhythmicity.