The study aims to evaluate subjects with gAChR-Ab positive (ganglionic acetylcholine receptor Ab) autoimmune ganglionopathy with multi-modal testing to understand the biomarkers of recovery and response.

Thirteen subjects with gAChR-Ab more than 100pM and autonomic failure were the population of the study. In 2018, the subjects were assessed with salivary, lacrimal, cardiovascular, urinary, pupillary, and sudomotor testing, including the COMPASS-31 questionnaire. The researchers calculated the ratio of orthostatic intolerance. Eleven of the subjects received immunotherapy.

The primary assessment showed all thirteen individuals with pupillary and cardiovascular impairments. Seven out of eight had a post-ganglionic sudomotor problem, nine of eleven had xeropthalmia and urinary retention, and six of eight had xerostomia. There was an improvement in the ratio of orthostatic intolerance after immunotherapy from 33.3 to 5.2. The heart rate response to deep breathing changed from 1.5 to 4.5. the pupillary constriction to light changed from 12 to 19. The saliva production altered from 0.01 to 0.08. The COMPASS-31 score changed from 52 to 17. Patients’ skin samples showed nerve fiber loss in the epidermis and dermal autonomic adnexa.

The patients showed significant improvement after the therapy. The autonomic biomarkers can help to assess initial deficits, treatment response, and guide therapeutic decisions.

Ref: https://onlinelibrary.wiley.com/doi/10.1002/ana.26018

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