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

Postural Tachycardia Syndrome Clinical Trial

— ProANS  

Official title:

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)

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04310644
• Other study ID #: 19-016
• Status: Recruiting
• Start date: September 15, 2019
• Est. completion date: September 15, 2029

Study information

• Verified date: June 2023
• Source: RWTH Aachen University
• Contact: Andrea Maier, physician
• Phone: +492418089600
• Email: ans-ambulanz[@]ukaachen.de
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 200
• Est. completion date: September 15, 2029
• Est. primary completion date: September 15, 2024
• Gender: All
• Age group: 18 Years to 80 Years

Eligibility

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

Related Conditions & MeSH terms

• Autonomic Neuropathy
• Ehlers-Danlos Syndrome
• Ehlers-Danlos Syndrome Hypermobility Type
• Nervous System Diseases
• Peripheral Nervous System Diseases
• Postural Orthostatic Tachycardia Syndrome
• Postural Tachycardia Syndrome
• Small Fiber Neuropathy
• Syndrome
• Tachycardia

Intervention

Other:
• No intervention planned, but all patients get our standart treatment
Patients are selected from clinical Routine and get our standart Treatment based on their disease

Locations

Country	Name	City	State
Germany	University clinic RWTH Aachen	Aachen	Nordrhein Westfalen

Sponsors (2)

Lead Sponsor	Collaborator
RWTH Aachen University	DLR German Aerospace Center

Country where clinical trial is conducted

Germany, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Blood pressure	Change in systolic and diastolic blood pressure over time	baseline, 3 months and 18 months
Primary	Heart frequency	Change in heart frequency over time	baseline, 3 months and 18 months
Primary	Skin biopsy	intraepithelial nerve fiber density between groups	baseline
Primary	Composite autonomic severity Score	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)	baseline, 3 months and 18 months

External Links

•   official homepage of our outpatient clinic for autonomic nervous system disorders
•   Paper Skin biopsy reveals generalized small fibre neuropathy in hypermobile Ehlers-Danlos syndromes