Investigation of Autonomic Dysfunction in Patients With Familial Mediterranean Fever

Browse trials for

• Familial Mediterranean Fever 73
• Primary Dysautonomias 183

Keywords

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

Familial Mediterranean Fever

73 studies on the registry are indexed under Familial Mediterranean Fever; 28 are open to participants now.

This study's planned enrollment of 60 is below the median of 84 across 33 observational studies indexed under Familial Mediterranean Fever.

Lead sponsor

Marmara University is the lead sponsor of 590 studies on the registry; 135 are open to participants now.

Study population

Groups and cohorts

• Patients diagnosed with Familial Mediterranean Fever

  Diagnostic Test: Composite Autonomic Symptom Score-31 · Diagnostic Test: r-r interval variability with normal breathing · Diagnostic Test: r-r interval variability with deep breathing · Diagnostic Test: Valsalva ratio · Diagnostic Test: 30:15 ratio · Diagnostic Test: sympathetic skin response · Diagnostic Test: blood pressure response to standing · Diagnostic Test: blood pressure response to sustained handgrip test · Other: Hospital anxiety and depression scale · Diagnostic Test: Fibromyalgia rapid screening tool · Other: Familial Mediterranean Fever Quality of life scale · Other: Jenkins Sleep Evaluation Scale · Other: The Fatigue Severity Scale

• Healthy Controls

  Diagnostic Test: Composite Autonomic Symptom Score-31 · Diagnostic Test: r-r interval variability with normal breathing · Diagnostic Test: r-r interval variability with deep breathing · Diagnostic Test: Valsalva ratio · Diagnostic Test: 30:15 ratio · Diagnostic Test: sympathetic skin response · Diagnostic Test: blood pressure response to standing · Diagnostic Test: blood pressure response to sustained handgrip test · Other: Hospital anxiety and depression scale · Diagnostic Test: Fibromyalgia rapid screening tool · Other: Jenkins Sleep Evaluation Scale · Other: The Fatigue Severity Scale

Interventions

• Diagnostic testComposite Autonomic Symptom Score-31

  Standardized questionnaire to determine the level of autonomic dysfunction

  Also known as: COMPASS-31

• Diagnostic testr-r interval variability with normal breathing

  electrophysiological test to detect parasympathetic dysfunction

• Diagnostic testr-r interval variability with deep breathing

  electrophysiological test to detect parasympathetic dysfunction

• Diagnostic testValsalva ratio

  electrophysiological test to detect parasympathetic dysfunction

• Diagnostic test30:15 ratio

  electrophysiological test to detect parasympathetic dysfunction

• Diagnostic testsympathetic skin response

  electrophysiological test to detect sympathetic dysfunction

  Also known as: SSR

• Diagnostic testblood pressure response to standing

  clinical test to detect sympathetic dysfunction

• Diagnostic testblood pressure response to sustained handgrip test

  clinical test to detect sympathetic dysfunction

• OtherHospital anxiety and depression scale

  Standardized questionnaire to investigate the depression and anxiety

  Also known as: HADS

• Diagnostic testFibromyalgia rapid screening tool

  Standardized questionnaire to determine the presence of fibromyalgia

  Also known as: FIRST

• OtherFamilial Mediterranean Fever Quality of life scale

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

  Also known as: FMFQoL

• OtherJenkins Sleep Evaluation Scale

  Standardized questionnaire to investigate the sleep quality and disturbance

• OtherThe Fatigue Severity Scale

  Standardized questionnaire to investigate the fatigue severity

  Also known as: FSS

Primary outcomes

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.

   Time frame: 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.

   Time frame: 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.

   Time frame: 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.

   Time frame: 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.

   Time frame: 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.

   Time frame: 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.

   Time frame: 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.

   Time frame: 6 months

Secondary outcomes

1. Familial Mediterranean Fever Quality of Life Scale (FMF-QoL)

   FMF-QoL was developed to evaluate the quality of life in FMF patients. This scale consists of 20 questions in the form of a Likert scale and the total scoring is between 0-80. High scores indicate a decrease in quality of life.

   Time frame: 6 months

2. Hospital Anxiety and Depression Scale (HADS)

   This scale consists of 14 questions in total, and anxiety symptoms are questioned in half of the questions and depression-related complaints in the other half. A subscore of 8 or higher for depression or anxiety is considered a clinical case.

   Time frame: 6 months

3. Fibromyalgia Rapid Screening Tool (FIRST)

   This scale consists of 6 questions investigating the most relevant clinical features of fibromyalgia. The questions are answered as yes/no and 5 or more out of 6 points in total are in favor of fibromyalgia.

   Time frame: 6 months

4. The Fatigue Severity Scale (FSS)

   It consists of a total of 9 questions in which complaints related to fatigue are scored between 0 and 7, taking into account the fatigue in the last week. The mean score is obtained by dividing the total score by 9, and it is defined as \<4.0 points as no fatigue, 4.0-5.0 points at the border, and \>5.0 points as fatigue.

   Time frame: 6 months

5. Jenkins Sleep Evaluation Scale (JSS)

   It is a scale in which scores between 0 and 5 are made for each symptom by questioning how many days in a month 4 sleep-related symptoms are experienced considering the last 1 month. The total score is between 0-20, and as the score increases, sleep quality deteriorates.

   Time frame: 6 months

Individual participant data

Plan to share: No