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VBCR - October 2016, Vol 5, No 5 - Rheumatic Diseases
Wayne Kuznar

Hypermobility syndrome represents a complex clinical picture that is often neglected by health professionals, said Rodney Grahame, MD, Consultant Rheumatologist, The Hypermobility Unit at the Hospital of St. John and St. Elizabeth, London, England, at the 9th Annual Rheumatology Nurses Society Conference.

Enhanced pain perception and proprioceptive impairment are 2 of the neurologic and physiologic defects more recently identified in hypermobility syndrome; enhanced musculoskeletal pain is experienced by approximately two-thirds of patients with the condition.

Hypermobility syndrome is mostly predominant among young, highly motivated people in the performing arts whose function deteriorates, and “who are literally cut down in the prime of their life,” he said. The prevalence has been estimated at 3% of the population, or approximately 45% of patients who present to a rheumatology clinic, using the Beighton criteria.

Only approximately 5% of actual cases of hypermobility syndrome are recognized, with the remainder undiagnosed, and, presumably, untreated. The syndrome is often misdiagnosed as fibromyalgia, seronegative arthropathy, psychogenic rheumatism, or depression.

There is no genetic marker or diagnostic test, and “this means that we can’t verify the diagnosis in the laboratory, which is a bit of a handicap,” said Dr Grahame. “Because we can’t do that, people are questioning whether what we’re talking about is real.”

The diagnosis, therefore, is one made by recognition of the clinical manifestations, which include an excessive range of joint movement (taking into account age, sex, and ethnic background), unstable joints, joint pain, gastrointestinal dysmotility, and other extra-articular manifestations (eg, syncope and orthostatic hypotension), he said.

The early history also includes hip dysplasia, delayed walking, and clumsiness/falls, whereas noninflammatory joint pain, spinal pain, joint instability, and anxiety tend to appear later.

The 9-point Beighton hypermobility scale has limitations and is “overinterpreted,” according to Dr Grahame. “It tells you nothing about the degree of hypermobility; all it does, is…flag up the fact that a certain number of joints out of a very limited selection are hypermobile,” he said. Evaluating the mobility of joints outside the Beighton scale (eg, hips, shoulders, and thoracic spine) is important.

Skin in patients with hypermobility syndrome is often semitransparent and stretchy. The triad of joint hypermobility symptoms, increased stretch in the skin, and Marfanoid features is diagnostic.

Too often, the pain associated with hypermobility is dismissed as being psychogenic. In addition to a precise diagnosis, hypermobility syndrome requires a multifaceted program that incorporates education, pain management, and physical rehabilitation. Establishing a rapport with the patient is essential to earning trust.

“Remember that conventional therapeutic approaches are often disappointing,” said Dr Grahame. Rehabilitation should focus on developing core stability, enhancing joint stability, improving joint proprioception, and reversing any deconditioning that may have occurred as a result of inadequate pain management. Advise patients to avoid resting in end-of-range postures. The multidisciplinary management team should include physical therapists, occupational therapists, podiatrists, nurses, and pain psychologists.

An 8-week home-based program of closed kinetic chain exercises has been shown to improve proprioception, balance, muscle strength, and quality of life. Pain management programs that employ cognitive behavioral techniques can also help to reduce the effects of hypermobility on patients’ lives, and can reduce pain-related distress, especially fear.

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Last modified: November 7, 2016
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