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Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome

Inge De Wandele (UGent) , Lies Rombaut (UGent) , Tine De Backer (UGent) , Wim Peersman (UGent) , Hellen Da Silva Van den Bosch (UGent) , Sophie De Mits (UGent) , Anne De Paepe (UGent) , Patrick Calders (UGent) and Fransiska Malfait (UGent)
(2016) RHEUMATOLOGY. 55(8). p.1412-1420
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Abstract
OBJECTIVE: To investigate whether orthostatic intolerance (OI) is a significant predictor for fatigue in Ehlers-Danlos Syndrome, hypermobility type (EDS-HT). METHODS: Eighty patients with EDS-HT and 52 controls participated in the first part of the study, which consisted of questionnaires. Fatigue was evaluated using the Checklist Individual Strength (CIS). As possible fatigue determinants OI [Autonomic Symptom Profile (ASP)], habitual physical activity (Baecke), affective distress [Hospital Anxiety and Depression Scale (HADS)], pain (SF36), medication use and generalized hypermobility (5-point score of Grahame and Hakim regarding generalized joint hypermobility) were studied. Next, a 20 min head-up tilt (70°) was performed in a subsample of 39 patients and 35 controls, while beat-to-beat heart rate and blood pressure were monitored (Holter, Finometer Pro). Before and after tilt, fatigue severity was assessed using a numeric rating scale. RESULTS: Patients scored significantly higher on the CIS [total score: EDS: 98.2 (18.63)vscontrols: 45.8 (16.62), P < 0.001] and on the OI domain of the ASP [EDS: 22.78 (7.16)vscontrols: 6.5 (7.78)]. OI was prevalent in EDS-HT (EDS: 74.4%, controls: 34.3%, P = 0.001), and frequently expressed as postural orthostatic tachycardia (41.0% of the EDS group). Patients responded to tilt with a higher heart rate and lower total peripheral resistance (p < 0.001; p = 0.032). This altered response correlated with fatigue in daily life (CIS). In the EDS-HT group, tilt provoked significantly more fatigue [numeric rating scale increase: EDS: +3.1 (1.90), controls: +0.5 (1.24), P < 0.001]. Furthermore, the factors OI, pain, affective distress, decreased physical activity and sedative use explained 47.7% of the variance in fatigue severity. CONCLUSION: OI is an important determinant of fatigue in EDS-HT.
Keywords
head-up tilt, hypermobility, fatigue, Ehlers-Danlos syndrome, orthostatic intolerance, cardiovascular dysautonomia, NEURALLY-MEDIATED HYPOTENSION, POSTURAL TACHYCARDIA SYNDROME, CONSENSUS STATEMENT, PHYSICAL-ACTIVITY, BRAIN FOG, QUESTIONNAIRE, DEFINITION, SYMPTOMS, MIDODRINE, MARFAN

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Chicago
De Wandele, Inge, Lies Rombaut, Tine De Backer, Wim Peersman, Hellen Da Silva Van den Bosch, Sophie De Mits, Anne De Paepe, Patrick Calders, and Fransiska Malfait. 2016. “Orthostatic Intolerance and Fatigue in the Hypermobility Type of Ehlers-Danlos Syndrome.” Rheumatology 55 (8): 1412–1420.
APA
De Wandele, I., Rombaut, L., De Backer, T., Peersman, W., Da Silva Van den Bosch, H., De Mits, S., De Paepe, A., et al. (2016). Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome. RHEUMATOLOGY, 55(8), 1412–1420.
Vancouver
1.
De Wandele I, Rombaut L, De Backer T, Peersman W, Da Silva Van den Bosch H, De Mits S, et al. Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome. RHEUMATOLOGY. 2016;55(8):1412–20.
MLA
De Wandele, Inge, Lies Rombaut, Tine De Backer, et al. “Orthostatic Intolerance and Fatigue in the Hypermobility Type of Ehlers-Danlos Syndrome.” RHEUMATOLOGY 55.8 (2016): 1412–1420. Print.
@article{7205617,
  abstract     = {OBJECTIVE: To investigate whether orthostatic intolerance (OI) is a significant predictor for fatigue in Ehlers-Danlos Syndrome, hypermobility type (EDS-HT).
METHODS: Eighty patients with EDS-HT and 52 controls participated in the first part of the study, which consisted of questionnaires. Fatigue was evaluated using the Checklist Individual Strength (CIS). As possible fatigue determinants OI [Autonomic Symptom Profile (ASP)], habitual physical activity (Baecke), affective distress [Hospital Anxiety and Depression Scale (HADS)], pain (SF36), medication use and generalized hypermobility (5-point score of Grahame and Hakim regarding generalized joint hypermobility) were studied. Next, a 20 min head-up tilt (70{\textdegree}) was performed in a subsample of 39 patients and 35 controls, while beat-to-beat heart rate and blood pressure were monitored (Holter, Finometer Pro). Before and after tilt, fatigue severity was assessed using a numeric rating scale.
RESULTS: Patients scored significantly higher on the CIS [total score: EDS: 98.2 (18.63)vscontrols: 45.8 (16.62), P {\textlangle} 0.001] and on the OI domain of the ASP [EDS: 22.78 (7.16)vscontrols: 6.5 (7.78)]. OI was prevalent in EDS-HT (EDS: 74.4\%, controls: 34.3\%, P = 0.001), and frequently expressed as postural orthostatic tachycardia (41.0\% of the EDS group). Patients responded to tilt with a higher heart rate and lower total peripheral resistance (p {\textlangle} 0.001; p = 0.032). This altered response correlated with fatigue in daily life (CIS). In the EDS-HT group, tilt provoked significantly more fatigue [numeric rating scale increase: EDS: +3.1 (1.90), controls: +0.5 (1.24), P {\textlangle} 0.001]. Furthermore, the factors OI, pain, affective distress, decreased physical activity and sedative use explained 47.7\% of the variance in fatigue severity.
CONCLUSION: OI is an important determinant of fatigue in EDS-HT.},
  author       = {De Wandele, Inge and Rombaut, Lies and De Backer, Tine and Peersman, Wim and Da Silva Van den Bosch, Hellen and De Mits, Sophie and De Paepe, Anne and Calders, Patrick and Malfait, Fransiska},
  issn         = {1462-0324},
  journal      = {RHEUMATOLOGY},
  keyword      = {head-up tilt,hypermobility,fatigue,Ehlers-Danlos syndrome,orthostatic intolerance,cardiovascular dysautonomia,NEURALLY-MEDIATED HYPOTENSION,POSTURAL TACHYCARDIA SYNDROME,CONSENSUS STATEMENT,PHYSICAL-ACTIVITY,BRAIN FOG,QUESTIONNAIRE,DEFINITION,SYMPTOMS,MIDODRINE,MARFAN},
  language     = {eng},
  number       = {8},
  pages        = {1412--1420},
  title        = {Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome},
  url          = {http://dx.doi.org/10.1093/rheumatology/kew032},
  volume       = {55},
  year         = {2016},
}

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