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

• Sponsor: RWTH Aachen University (Other)
• Overall Status: Recruiting
• CT.gov ID: NCT04310644
• Collaborator: DLR German Aerospace Center (Other)
• Enrollment: 200
• Location: 1
• Anticipated Duration (Months): 120
• Patients Per Site Per Month: 1.7

Study Details

Study Description

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.

Condition or Disease	Intervention/Treatment	Phase
Autonomic Neuropathy; Ehlers-Danlos Syndrome Hypermobility Type; Small Fiber Neuropathy; Postural Tachycardia Syndrome	Other: No intervention planned, but all patients get our standart treatment

Detailed Description

Inclusion of patients with autoimmune autonomic neuropathies/ pure autonomic failure, postural orthostatic tachycardia syndrome, small fiber neuropathies and Ehlers Danlos syndromes in our Register study with follow up visits. Comparison to healthy controls.

Planned examinations are laboratory tests, questionnaires on mental and physical health status and circulatory disorders, attention tests, tilt table testing, 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.

Study Design

• Study Type: Observational [Patient Registry]
• Anticipated Enrollment: 200 participants
• Observational Model: Case-Control
• Time Perspective: Prospective
• 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?
• Actual Study Start Date: Sep 15, 2019
• Anticipated Primary Completion Date: Sep 15, 2024
• Anticipated Study Completion Date: Sep 15, 2029

Arms and Interventions

Arm	Intervention/Treatment
Postural Orthostatic Tachycardia Syndrome Patients with orthostatic intolerance because of Postural orthostatic tachycardia syndrome diagnosed in our outpatient clinic by tilt table examination.	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
Ehlers Danlos Syndrome Patients with hypermobile or classical EDS who are already diagnosed including genetical testing for classical or vascular EDS and Marfan Syndromes	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
Autoimmune autonomic neuropathy/Pure autonomic failure Patients who have an autoimmune autonomic neuropathy based on clinical diagnosis and antibody testing in our outpatient clinic. Cardial MIBG Scintigraphy should have been performed.	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
Healthy controls Healthy controls with no documented cardiovascular or neurological disorders and no symtoms of autonomic failure/dizziness/fainting

Outcome Measures

Primary Outcome Measures

1. Blood pressure [baseline, 3 months and 18 months]

   Change in systolic and diastolic blood pressure over time

2. Heart frequency [baseline, 3 months and 18 months]

   Change in heart frequency over time

3. Skin biopsy [baseline]

   intraepithelial nerve fiber density between groups

4. 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)

Eligibility Criteria

Criteria

• Ages Eligible for Study: 18 Years to 80 Years
• Sexes Eligible for Study: All

Inclusion Criteria:

• autonomic neuropathy

• Postural orthostatic tachycardia syndrome

• hypermobile or classical Ehlers Danlos syndromes

• 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

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
1	University clinic RWTH Aachen	Aachen	Nordrhein Westfalen	Germany	52074

Sponsors and Collaborators

• RWTH Aachen University
• DLR German Aerospace Center

Investigators

• Principal Investigator: Andrea Maier, University Hospital, Aachen

Study Documents (Full-Text)

None provided.

More Information

Additional Information:

• official homepage of our outpatient clinic for autonomic nervous system disorders

Publications

None provided.

• Responsible Party: RWTH Aachen University
• ClinicalTrials.gov Identifier: NCT04310644
• Other Study ID Numbers: 19-016
• First Posted: Mar 17, 2020
• Last Update Posted: May 10, 2022
• Last Verified: May 1, 2022

• Individual Participant Data (IPD) Sharing Statement: Undecided
• Plan to Share IPD: Undecided
• Studies a U.S. FDA-regulated Drug Product: No
• Studies a U.S. FDA-regulated Device Product: No
• Additional relevant MeSH terms: Peripheral Nervous System Diseases; Nervous System Diseases; Postural Orthostatic Tachycardia Syndrome; Small Fiber Neuropathy; Tachycardia; Ehlers-Danlos Syndrome; Syndrome

Study Results

No Results Posted as of May 10, 2022