Severe treatment-refractory antibody positive autoimmune autonomic ganglionopathy after mRNA COVID19 vaccination

• Published in: Autoimmunity reviews 2022-12, Vol.21 (12), p.103201, Article 103201
• Main Authors: Rowe, Simon, Spies, Judith M., Urriola, Nicolás
• Format: Article
• Language: English
• Subjects: Autoimmune autonomic ganglionopathy; Case report; Comirnaty; COVID19 vaccination; mRNA vaccine; Pandysautonomia
• ISSN: 1568-9972; 1568-9972; 1873-0183
• DOI: 10.1016/j.autrev.2022.103201

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. 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. 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.