关键词: Autoimmune autonomic ganglionopathy COVID19 vaccination Case report Comirnaty Pandysautonomia mRNA vaccine

Mesh : Male Humans Middle Aged COVID-19 Vaccines / adverse effects Ganglia, Autonomic RNA, Messenger COVID-19 Autoimmune Diseases Autonomic Nervous System Diseases / etiology diagnosis Primary Dysautonomias / etiology Peripheral Nervous System Diseases Autoantibodies Autoimmune Diseases of the Nervous System / etiology diagnosis

来  源:   DOI:10.1016/j.autrev.2022.103201

Abstract:
BACKGROUND: COVID-19 vaccine-associated peripheral and central neuroimmunological disorders have been well described. We present the case of a 56 year old male who developed α3-ganglionic AChR antibody positive Autoimmune Autonomic Ganglionopathy (AAG) after completion of a two-dose course of mRNA (Comirnaty) vaccination for COVID19.
RESULTS: A previously hypertensive 56 year old male presented with the subacute onset of severe constipation, urinary retention, erectile dysfunction, sudomotor failure, sicca symptoms, non-reactive pupils and severe orthostatic hypotension shortly after receiving the second dose of an mRNA vaccine against COVID19. Autonomic testing revealed severe cardiovagal, adrenergic and sudomotor impairment, and tonic \'half-mast\' pupils with evidence of sympathetic and parasympathetic denervation. Pathological α3-ganglionic ACHR antibodies were positive in serum as detected by a new flow cytometric immunomodulation assay. Malignancy was excluded. The patient was diagnosed with severe, treatment-refractory acute AAG.
CONCLUSIONS: While autonomic dysfunction has been previously reported post-COVID19 vaccination, to our knowledge this is the first reported case of antibody-positive AAG in this setting. The severity of this case is in marked contrast to the existing literature on idiopathic antibody-positive autoimmune pandysautonomia.
摘要:
背景:COVID-19疫苗相关的外周和中枢神经免疫疾病已得到充分描述。我们介绍了一名56岁的男性,该男性在完成COVID19的两剂量mRNA(Comirnaty)疫苗接种后,出现了α3-神经节AChR抗体阳性的自身免疫性自主神经神经节病(AAG)。
结果:一名56岁男性高血压患者出现亚急性严重便秘,尿潴留,勃起功能障碍,sudomotor失败,干燥症状,接受第二剂针对COVID19的mRNA疫苗后不久,非反应性瞳孔和严重的直立性低血压。自主测试显示严重的心迷走神经,肾上腺素能和sudomotor损伤,和补品“半肥大”瞳孔,有交感神经和副交感神经支配的证据。通过新的流式细胞术免疫调节试验检测到,血清中的病理性α3-神经节ACHR抗体呈阳性。恶性肿瘤被排除。病人被诊断为严重的,治疗难治性急性AAG。
结论:虽然先前已经报道了COVID19疫苗接种后的自主神经功能障碍,据我们所知,这是在这种情况下报告的首例抗体阳性AAG病例。这种情况的严重程度与现有的特发性抗体阳性的自身免疫性自主神经病的文献形成鲜明对比。
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