Autonomic Small Fiber Neuropathy and Ehlers Danlos Syndromes - Prospective Study and Registry

Autonomic Neuropathy and Its Pathophysiology in Autoimmune Autonomic Neuropathies, Postural Orthostatic Tachycardia Syndrome and Ehlers Danlos Syndromes: Peripheral Autonomic Small Fiber Neuropathy or Central Autonomic Failure? Validation of the Malmö POTS Score and a Better Diagnosis of POTS in Patientes With Autnomic Failure (VaGeMAPS-3-step-Diag)

Status

Recruiting

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT04310644

Acronym

ProANS

Enrollment

200

Registered

2020-03-17

Start date

2019-09-15

Completion date

2029-09-15

Last updated

2025-02-04

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Autonomic Neuropathy, Ehlers-Danlos Syndrome Hypermobility Type, Postural Tachycardia Syndrome, Small Fiber Neuropathy

Brief summary

We examine patients with different autonomic neuropathies and Ehlers Danlos syndromes compared to healthy controls at three different points over time (baseline, after 3 months and after 1.5 years) to gain knowledge about the course of this disease and understand its pathophysiology, with a focus on Small Fiber neuropathy. Moreover we will validate the german version of the Malmö POTS Score and establish an easy diagnostic scheme for patients in outpatient care.

Detailed description

Inclusion of patients with autoimmune autonomic neuropathies/ pure autonomic failure, postural orthostatic tachycardia syndrome, small fiber neuropathies, Ehlers-Danlos syndromes, Mast cell activation syndrom, Chronic fatigue syndrome and PostCOVID syndrome in our Registry study with follow up visits. Comparison to healthy controls concerning selected examinations. Planned examinations are laboratory tests, questionnaires on mental and physical health status and circulatory disorders, attention tests, tilt table testing, standing test, sweat function, investigation of small fiber function via quantitative sensory testing as well as the density of nerve fibers in the skin. Measurements are performed at baseline mostly in clinical routine and follow up visits are offered.

Interventions

OTHERNo intervention planned, but all patients get our standart treatment

Patients are selected from clinical Routine and get our standart Treatment based on their disease

Study design

Observational model

CASE_CONTROL

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

18 Years to 80 Years

Inclusion criteria

* autonomic neuropathy * Postural orthostatic tachycardia syndrome * hypermobile or classical Ehlers Danlos syndromes * Chronic fatigue syndrome, mast cell activation syndrome and/or PostCOVID * healty controls * between 18-80 years * in patients: diagnosis and clinical testing in our outpatient clinic * German speaking

Exclusion criteria

* pregnancy * Pacemaker or Deep brain Stimulation * sensory or motor Polyneuropathy * neurodegenerative disease

Design outcomes

Primary

Measure	Time frame	Description
Blood pressure	baseline, 3 months and 18 months	Change in systolic and diastolic blood pressure over time
Heart frequency	baseline, 3 months and 18 months	Change in heart frequency over time
Skin biopsy	baseline	intraepithelial nerve fiber density between groups
Composite autonomic severity Score	baseline, 3 months and 18 months	Change of the Score over time, the results are interpreted as normal (score=0), mild (score=1-3), moderate (score=4-6) or severe (score=7-10)

Countries

Germany

Contacts

Primary ContactAndrea Maier, physician

ans-ambulanz@ukaachen.de+492418089600

Backup ContactDenver Igharo, student