Immunomodulatory treatment in postural tachycardia syndrome: A case series.

Rodriguez, Belén; Hoepner, Robert; Salmen, Anke; Kamber, Nicole; Z'Graggen, Werner J. (2021). Immunomodulatory treatment in postural tachycardia syndrome: A case series. European journal of neurology, 28(5), pp. 1692-1697. Wiley 10.1111/ene.14711

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BACKGROUND AND PURPOSE

Postural tachycardia syndrome (POTS) is a form of autonomic dysfunction characterized by symptoms of orthostatic intolerance, often accompanied by sudomotor dysfunction and gastrointestinal dysmotility. Recently, evidence has accumulated that in a subset of patients, the pathogenesis of dysautonomia may be immune-mediated. The aim of the current report was to evaluate the use of intravenous immunoglobulin (IVIG) treatment in patients with progressive and/or refractory immune-mediated POTS.

METHODS

We retroactively assessed the effect and tolerance of monthly administered IVIG in six patients using autonomic function testing, standardized symptom questionnaires, and patients' symptom diaries both before and 6 months into IVIG treatment. Objective outcome measures included heart rate increase after 10 min of head-up tilt as well as duration and anhidrotic area in a thermoregulatory sweat test. Subjective outcome measures were patient reports and symptom ratings from the symptom questionnaire.

RESULTS

All patients responded to immunomodulatory treatment, regardless of disease duration. After 6 months of IVIG, symptom severity was reduced by nearly 40%. Autonomic function testing showed improved cardiovascular functioning by 50% and a reduction of anhidrotic areas by one third. Overall, tolerance of IVIG treatment was poor, but could be improved by a reduction in infusion rate, premedication with steroids, and additional intravenous hydration.

CONCLUSIONS

Using subjective but also standardized objective measures, the case series describes promising effects of IVIG treatment in POTS patients with immune-mediated dysautonomia. By reducing the infusion rate, pretreatment with steroids, and intravenous hydration, tolerance could be improved, and no patient had to discontinue the treatment.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurosurgery
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

Graduate School:

Graduate School for Health Sciences (GHS)

UniBE Contributor:

Rodriguez Galdin, Belén; Hoepner, Robert; Salmen, Anke; Kamber, Nicole and Z'Graggen, Werner Josef

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1468-1331

Publisher:

Wiley

Language:

English

Submitter:

Belén Rodriguez

Date Deposited:

10 Mar 2021 11:47

Last Modified:

17 Apr 2021 01:34

Publisher DOI:

10.1111/ene.14711

PubMed ID:

33382525

Uncontrolled Keywords:

alpha-1 adrenergic receptor autoantibodies autoimmune disturbance of sweating gastroparesis muscarinic cholinergic receptors orthostatic intolerance

BORIS DOI:

10.48350/153349

URI:

https://boris.unibe.ch/id/eprint/153349

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