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Joint Hypermobility Syndrome and Postural Orthostatic Tachycardia Syndrome (HyPOTS)

Dana Mandel

Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine, University Hospitals Cleveland Medical Center, USA

E-mail : aa

Ali D. Askari

Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine, University Hospitals Cleveland Medical Center, USA

Charles J. Malemud

Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine, University Hospitals Cleveland Medical Center, USA

Artan Kaso

Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine, University Hospitals Cleveland Medical Center, USA

DOI: 10.15761/BRCP.1000132

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Abstract

Objective: To evaluate the association between joint hypermobility syndrome associated with postural orthostatic tachycardia syndrome (HyPOTS) and fibromyalgia.

Methods: A chart review of patients followed at two outpatient Rheumatology facilities within one hospital system were analyzed.  Thirty-seven patients (3 male, 34 female) diagnosed with HyPOTS and fibromyalgia were reviewed.

Results: HyPOTS is a chronically disabling musculoskeletal disorder presenting clinically as widespread musculoskeletal pain and/or fatigue with joint hypermobility. However, HyPOTS may be inadvertently diagnosed as chronic fatigue syndrome or fibromyalgia. In fact, the group of fibromyalgia patients evaluated at a rheumatology outpatient clinic were found to meet the clinical criteria for HyPOTS.

Conclusions: Patients diagnosed with fibromyalgia and chronic fatigue syndrome may benefit from a further evaluation for HyPOTS. Thus, if HyPOTS is properly defined, a wide range of therapeutic options for these patients become available in order to improve their physical manifestations and quality of life.

Key words

HyPOTS, fibromyalgia,

Introduction

Often, the most common complaints voiced by patients in a rheumatology office are that of increased arthralgias, myalgias, and constitutional symptoms.  It is not uncommon to receive a referral for evaluation of a patient with complaints of arthralgias, myalgias and other constitutional symptoms but without obvious signs of synovitis or connective tissue disease.  It is also common to have these complaints attributed to patient’s underlying disease due to their nonspecific nature.  However, nonspecific arthralgias, myalgias, and fatigue are just that – nonspecific.  Yet, these symptoms can lead to significant decline in a patient’s quality of life. As physicians, we consistently strive to treat disease and to improve a patient’s quality of life.  This may mean prescribing a treatment strategy, including immunosuppressive therapy, and taking on the risk of increased side-effects.  We propose that in select patients, joint hypermobility may be contributing to these non-specific complaints and that recognition and intervention can help to improve the patient’s quality of life.

Joint hypermobility syndrome (JHS) is a chronic, disabling disorder which manifests as widespread musculoskeletal pain and/or fatigue in the presence of generalized joint hypermobility. Joint hypermobility can exist without any associated musculoskeletal disorder.  Although a limited literature is available on joint hypermobility; approximately 5% of the adult Caucasian population has JHS with an increased prevalence noted in Asian and African-American populations [1].  Furthermore, the prevalence of JHS is noted to be approximately 10-30% in the juvenile population which decreases with age [2].  Despite the fact that there is a limited understanding of the pathophysiology of JHS, genetics do appear to play a role.  For example, in studies performed on the expression of the tenascin-X gene, the protein product of the gene has been noted to be present in low amounts in patients diagnosed with JHS and the Ehlers-Danlos Syndrome (hypermobility type) [2].  Furthermore, environmental triggers, such as viral illness or extended periods of deconditioning, have been proposed as causes for benign joint hypermobility transitioning into JHS [2].

In addition to musculoskeletal pain and fatigue, a subset of patients with JHS have also been found to suffer from visceral manifestations and postural tachycardia with orthostatic intolerance.   Visceral manifestations often center upon gastrointestinal dysfunction.  Of note, abnormalities in proprioception have also been observed in