1 Centre for Rheumatology, University College London Hospitals and 2 Academic Rheumatology and Osteoporosis Unit, Whipps Cross University Hospital, London, UK
Correspondence to: A. J. Hakim, Whipps Cross University Hospital, Leytonstone, London E11 1NR, UK. E-mail: alanhakim{at}aol.com
SIR, Joint hypermobility syndrome (JHS) is a chronically disabling disorder manifested as widespread pain, fatigue, multiple soft tissue lesions and fragility of skin and supportive connective tissues [1]. It is a condition that is often overlooked by clinicians [2]. Moreover, clinical experience suggests that previously unrecognized non-musculoskeletal symptoms, including presyncope, palpitations and bowel disturbance, are also common in JHS. Recent evidence demonstrates dysfunction of the autonomic nervous system as an explanation for these symptoms [3]. Recognition of these symptoms by clinicians is an important part of patient assessment and education, even if the pathophysiology remains unclear.
We have examined the prevalence of non-musculoskeletal complaints and explored their associations to determine whether they reflect a tendency to report multiple, non-specific concerns.
One hundred and seventy women aged between 18 and 65 yr were seen in a teaching hospital hypermobility clinic over a 2-yr period. Each was diagnosed with JHS using the 1998 Brighton criteria [4]. Individuals completed a self-reported questionnaire enquiring about symptoms experienced on a regular basis. The questionnaire was structured so that patients were unaware of any hypothesis. Fifty female hospital staff acted as controls, having been identified as non-hypermobile by the use of a five-part self-report questionnaire [5]. The symptoms explored clustered into five domains: (i) (pre)syncope (feel faint, actually faint, dizziness and light-headedness); (ii) cardiorespiratory (CR) (palpitations, chest pain and shortness of breath); (iii) gastrointestinal (GI) (nausea, stomach ache, diarrhoea and constipation); (iv) common JHS concerns (fatigue, joint pain, anxiety and depression); and (v) non-specific (migraine, allergy, rash, nocturia, dysuria, flushing, night sweats, fever, lymph gland pain and poor sleep).
Similar symptoms were combined for analysis. For example, dizziness and light-headedness were considered synonymous with presyncope and were not treated as mutually exclusive. They were combined, so that a person giving both in their response was only counted once within the domain.
We found that 41, 26 and 37% of individuals with JHS reported at least one symptom suggestive of a (pre)syncopal, CR or GI complaint respectively. This compared with 15, 12 and 16% of controls, despite controls being older by a mean of 13 yr [32 yr (range 1865) vs 45 yr (range 2364)].
Pain, fatigue, anxiety and depression were, as one would expect, more common in JHS patients (91, 71, 32 and 38% respectively) than controls (30, 30, 12 and 8% respectively). Migraine, rashes and poor sleep were also over-represented amongst JHS patients. Other non-specific complaints (allergy, nocturia, dysuria, flushing, night sweats, fever and lymph gland pain) were equally distributed in cases and controls (Fig. 1).
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Potential manifestations of autonomic pathology include cardiac dysrhythmias, postural orthostatic tachycardia syndrome, orthostatic hypotension and orthostatic intolerance. Mechanisms leading to such phenomena in JHS patients may include weakened vascular tissue elasticity and impaired peripheral vasoregulation as a consequence of adrenoceptor or neuronal abnormalities. Similar symptoms are found in chronic fatigue syndrome [6]. Consequently, these disturbances might also be secondary and reflect a degree of physical deconditioning rather than a primary autonomic or connective tissue pathology. Further studies are required. In the meantime, clinical assessment should include an enquiry as to the presence of such symptoms, and health professionals should acknowledge that they are often encountered among patients with JHS.
Ethical approval for the use of a general questionnaire identifying features of JHS both in clinical and epidemiological studies was gained from Guy's Hospital, London. Verbal consent was deemed sufficient.
The authors have declared no conflicts of interest.
References