The Link between EDS and CFS/ME

Is The Physical Examination Normal in CFS? Part 2: Joint Hypermobility

In the 2nd part of this three-part blog series on physical examination in ME/CFS, Dr. Peter Rowe discusses joint hypermobility.

Some patients meeting the criteria for CFS have a genetic disorder of connective tissue known as Ehlers-Danlos syndrome (EDS). Those with EDS have chronic fatigue and widespread pain of uncertain cause, which of course overlaps with the central features of CFS.

We first drew attention to this association in 1999, noting that those with CFS and orthostatic intolerance had a much higher prevalence of EDS than expected.

Since the publication of these results, a number of studies have confirmed that those with joint hypermobility have more orthostatic symptoms and a reduced tolerance of upright posture, and that fatigue is a prominent contributor to lower quality of life in EDS. New work from investigators in Belgium confirms that those with hypermobile EDS have high rates of autonomic symptoms, abnormal heart rate responses to upright posture, and other features of autonomic dysfunction.

Why does the assessment for joint hypermobility matter? Joint hypermobility is associated with other co-morbid disorders such as temporomandibular joint dysfunction, and the laxity in ligaments often contributes to arthralgias and myalgias. Better ascertainment of these problems can lead to more focused therapy directed at pain, and physical treatments directed at biomechanical dysfunctions.

Beyond the effects on joints, connective tissue laxity also affects the blood vessel walls. The increased distensibility in blood vessels may be a factor in the early development of varicose veins in those with EDS. Vascular stretch also is thought to allow increased blood pooling in the dependent circulation during upright posture, leading to diminished blood return to the heart, and thus to orthostatic intolerance symptoms.

Given the high prevalence of skin and joint laxity in those with CFS, and the contribution of connective tissue abnormalities to the development of orthostatic intolerance, we recommend that all those with CFS undergo screening with a Beighton score, and that clinicians look carefully for the skin changes of EDS in their patients with CFS.