Seminars in Arthritis and Rheumatism
Volume 40, Issue 3 , Pages 267-274, December 2010

Fatigue Is a Frequent and Clinically Relevant Problem in Ehlers-Danlos Syndrome

  • Nicol C. Voermans, MD

      Affiliations

    • Neuromuscular Centre Nijmegen, Department of Neurology, Nijmegen, The Netherlands
    • Corresponding Author InformationAddress reprint requests to: N.C. Voermans, MD, Department of Neurology, 935, Donders Institute of Brain, Cognition and Behaviour, Radboud University Nijmegen Medical Centre, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands
    • ,
  • Hans Knoop, PhD

      Affiliations

    • Clinical psychologist, Expert Centre Chronic Fatigue, Nijmegen, The Netherlands
    • ,
  • Nicole van de Kamp, MSc

      Affiliations

    • Neuromuscular Centre Nijmegen, Department of Neurology, Nijmegen, The Netherlands
    • ,
  • Ben C. Hamel, MD, PhD

      Affiliations

    • Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    • ,
  • Gijs Bleijenberg, PhD

      Affiliations

    • Clinical psychologist, Expert Centre Chronic Fatigue, Nijmegen, The Netherlands
    • ,
  • Baziel G. van Engelen, MD, PhD

      Affiliations

    • Neuromuscular Centre Nijmegen, Department of Neurology, Nijmegen, The Netherlands

published online 02 November 2009.

Objectives

Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Fatigue and musculoskeletal pain are associated features but have never been studied systematically. We used a multidimensional assessment method to measure fatigue, its clinical relevance, and possible determinants.

Methods

A questionnaire study was performed among 273 EDS patients. The following dimensions were assessed: fatigue severity, functional impairment in daily life, physical activity, psychological distress, sleep disturbances, concentration problems, social functioning, self-efficacy concerning fatigue, causal attribution of fatigue, pain, and disease-related factors.

Results

More than three-quarters of EDS patients suffer from severe fatigue. Patients who are severely fatigued are more impaired than nonseverely fatigued patients and report a higher level of psychological distress. The 5 possible determinants involved in fatigue are sleep disturbances, concentration problems, social functioning, self-efficacy concerning fatigue, and pain severity.

Conclusions

This is the first study of fatigue and its possible determinants in EDS and shows that fatigue is a frequent and clinically significant problem in EDS. The 5 possible determinants of fatigue could form a starting point for the development of an effective cognitive behavioral intervention for fatigue in EDS.

Keywords: Ehlers-Danlos syndrome, heritable connective tissue disorder, fatigue, fatigue severity, questionnaire, pain, sleep disturbances, concentration, social functioning, self-efficacy

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 N.C. Voermans was supported by a research grant of the NWO (Netherlands Organization for Scientific Research). The NWO had no influence on the design or outcome of the study; hence, N.C. Voermans was independent of this sponsor. None of the authors have any conflicts of interest to disclose. All authors of the article had access to the original data, have fulfilled the criteria for authorship, and have read and approved the final version of the article.

PII: S0049-0172(09)00098-5

doi:10.1016/j.semarthrit.2009.08.003

Seminars in Arthritis and Rheumatism
Volume 40, Issue 3 , Pages 267-274, December 2010

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