Investigation of Autonomic Dysfunction in Patients With Familial Mediterranean Fever

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

• Ages 18 to 65 years
• All sexes
• Healthy volunteers accepted
• Adult, Older adult

Study population: Patients with FMF Healthy control group

Trial locations and contacts

1 site in 1 country.

About this study

Conditions

Familial Mediterranean Fever, Autonomic Dysfunction

Being tested

Composite Autonomic Symptom Score-31, r-r interval variability with normal breathing, r-r interval variability with deep breathing, Valsalva ratio, 30:15 ratio

Keywords

Familial Mediterranean Fever, Autonomic Dysfunction, r-r interval variability, sympathetic skin response

Medical subject headings

Familial Mediterranean Fever, Primary Dysautonomias

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

• Composite Autonomic Symptom Score-31Diagnostic test

  Also called COMPASS-31

  Standardized questionnaire to determine the level of autonomic dysfunction

• r-r interval variability with normal breathingDiagnostic test

  electrophysiological test to detect parasympathetic dysfunction

• r-r interval variability with deep breathingDiagnostic test

  electrophysiological test to detect parasympathetic dysfunction

• Valsalva ratioDiagnostic test

  electrophysiological test to detect parasympathetic dysfunction

• 30:15 ratioDiagnostic test

  electrophysiological test to detect parasympathetic dysfunction

• sympathetic skin responseDiagnostic test

  Also called SSR

  electrophysiological test to detect sympathetic dysfunction

• blood pressure response to standingDiagnostic test

  clinical test to detect sympathetic dysfunction

• blood pressure response to sustained handgrip testDiagnostic test

  clinical test to detect sympathetic dysfunction

• Hospital anxiety and depression scaleOther

  Also called HADS

  Standardized questionnaire to investigate the depression and anxiety

• Fibromyalgia rapid screening toolDiagnostic test

  Also called FIRST

  Standardized questionnaire to determine the presence of fibromyalgia

• Familial Mediterranean Fever Quality of life scaleOther

  Also called FMFQoL

  Standardized questionnaire to investigate the quality of life in Familial Mediterranean Fever patients

• Jenkins Sleep Evaluation ScaleOther

  Standardized questionnaire to investigate the sleep quality and disturbance

• The Fatigue Severity ScaleOther

  Also called FSS

  Standardized questionnaire to investigate the fatigue severity

What is being measured

The primary outcome is the main question the study is designed to answer.

1. Composite Autonomic Symptom Score-31

   It is a widely applicable, up-to-date, easy-to-apply and scientifically-based test that evaluates autonomic symptoms and functions by the person himself. It consists of 31 multiple-choice questions in 6 autonomic areas: orthostatic intolerance, vasomotor, secretomotor, gastrointestinal, bladder function and pupillomotor areas. A score in the range of 0-100 is obtained as a result of multiplying each area by the weight factor determined by the study. High score indicates presence and severity of autonomic dysfunction.

   Measured: 6 months

2. r-r interval variability with normal breathing

   The active and reference superficial recording electrodes are on the dorsal side of both hands, and the ground electrode is placed on the wrist. The R-R interval variability is calculated automatically by the electromyography device over a 1-minute recording.

   Measured: 6 months

3. r-r interval variability with deep breathing

   The active and reference superficial recording electrodes are on the dorsal side of both hands, and the ground electrode is placed on the wrist. In order to determine the R-R interval variability with deep breathing, the patient is given 6 to 8 deep breaths, consisting of 5 seconds of inspiration followed by 5 seconds of expiration, and the result is automatically calculated by the electromyography device.

   Measured: 6 months

4. Valsalva ratio

   The active and reference superficial recording electrodes are on the dorsal side of both hands, and the ground electrode is placed on the wrist. The test was performed by asking the subject to sit quietly and then blow into a mouthpiece attached to an aneroid pressure gauge at a pressure of 40 mmHg for 15 seconds. The ratio of the longest R-R interval shortly after the manoeuver (within about 20 beats) and the shortest R-R interval during the manoeuver is then measured. The result was expressed as the Valsalva ratio that is taken as the mean ratio from three successive Valsalva manoeuvers.

   Measured: 6 months

5. 30:15 ratio

   The active and reference superficial recording electrodes are on the dorsal side of both hands, and the ground electrode is placed on the wrist.The R-R interval on the ECG was recorded and used to determine the instantaneous HR at rest and then on the 15th and 30th beats after standing. The HR should normally rise after about 30 seconds as part of the response to return the blood pressure (BP) to normal. After standing up, the 30:15 ratio obtained by dividing the longest R-R distance around the 30th beat by the shortest R-R distance around the 15th beat was calculated automatically by the EMG device.

   Measured: 6 months

6. sympathetic skin response

   Sweating that occurs in response to many different stimuli, electrolyte in the skin triggers changes in skin conductivity, resulting in instantaneous changes in skin conductivity. Sympathetic skin response evaluates these instantaneous changes in the skin in response to sweating. Sympathetic skin response reflects the function of sympathetic nerve fibers.Active recording electrodes on palm and sole; reference electrodes is placed on the back of the hand and the back of the foot. Responses obtained by applying electrical current over the median nerve trace are evaluated.

   Measured: 6 months

7. blood pressure response to standing

   Participants were asked to stand up for 3 minutes after a 10-minute resting period in a supine position. The systolic and diastolic BP (SBP and DBP) just before standing, and 3 minutes after active standing were determined, in order to define postural change in BP and to evaluate orthostatic intolerance.

   Measured: 6 months

8. blood pressure response to sustained handgrip

   Blood pressure measurement is continued at 1-minute intervals while patients perform sustained handgip at 30% of their maximum volitional strength for 5 minutes.The absolute difference between the highest DBP during handgrip and the basal DBP just before the handgrip was noted.

   Measured: 6 months

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

1. First posted27 Oct 2022
2. Study start15 Mar 2022
3. Primary completion31 Oct 2022 (estimated)
4. Study completion31 Oct 2022 (estimated)

Resources

• Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
• Other trials for Familial Mediterranean FeverStudies of the same condition, open and under way.
• More trials run by Marmara UniversityThe sponsor's other registered studies.
• What the trial phases meanHow a treatment moves from first tests in people to approval.

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.