Severe autoimmune ganglionopathy after COVID19 vaccination

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 the Comirnaty 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 (see Figure). Tilt-table testing demonstrated a drop in blood pressure from 140/90 mmHg supine to 54/35 mmHg after two minutes at 60 degrees, with no heart rate response. There was no heart rate response to Valsalva maneuver and sympathetic skin responses in the hands and feet were absent. Sweat testing demonstrated complete anhidrosis.

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 Autoimmune Autonomic Ganglionopathy (AAG)

An incomplete remission has been achieved six months since illness-onset; while orthostatic symptoms have been mitigated, other features of autonomic failure remain present and severe.

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REFERENCE

Rowe S, Spies JM, Urriola N. Severe treatment-refractory antibody positive autoimmune autonomic ganglionopathy after mRNA COVID19 vaccination. Autoimmun Rev. 2022 Sep 20;21(12):103201. doi: 10.1016/j.autrev.2022.103201. Epub ahead of print. PMID: 36210629. 

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