ACR Meeting Report Ehlers-Danlos Syndrome and FM
AMERICAN COLLEGE OF RHEUMATOLOGY (ACR) MEETING REPORT
EHLERS-DANLOS SYNDROME AND FIBROMYALGIA
The last few NFMCPA Advocate Newsletters have contained articles as follow-up information from the November ACR meeting in Washington, DC. Most of the reports have been about abstracts that were presented at the meeting, but this article will focus on a presentation by Dr. Rodney Graham about Ehlers-Danlos Syndrome better known by the general public as joint-hypermobility. Dr. Graham is a rheumatologist from the U.K. who has devoted much of his career to this specific illness. His talk was an explanation of Ehlers-Danlos syndrome and some of its many complex symptoms.
Good physical examples of this condition are people considered to be “double jointed” such as contortionists, exceptional Ballet dancers who seem to accomplish super-human dance movements and ultra talented gymnasts. Obvious signs of joint-hypermobility are physical in nature, i.e. bending a thumb backward to touch a wrist; dislocating a joint repeatedly; various ways to do the “splits” and so on. These are outward signs of this syndrome which seem harmless while some of the more serious symptoms might concern the skin, digestive processes, and even mitral-valve prolapse a heart condition. Many of these symptoms are known to overlap with fibromyalgia.
Ehlers-Danlos syndrome (EDS) is named after two turn of the 20th century doctors, Edvard Ehlers from Denmark and Henri-Alexndre Danlos from France. It falls under a group of inherited connective tissue disorders, caused by a defect in the synthesis of collagen, naturally occurring proteins found in animals, especially in the flesh and connective tissues of vertebrates. Collagen in connective tissue helps tissues resist deformation. It plays a significant role in the skin, joints, muscles, ligaments, blood vessels and visceral organs; abnormal collagen leads to increased elasticity (stretchiness) within these structures. People who have this condition receive close monitoring of the digestive, excretory and particularly the cardiovascular systems. Physical therapy, bracing, and corrective surgery may help with the frequent injuries and pain that tend to develop in certain types of EDS, although extra caution and special practices are advised to prevent permanent damage. The pain associated with EDS is a serious complication and might be misdiagnosed as FM.
EDS symptoms associated with musculoskeletal conditions include:
- Hyper-flexible joints
- Unstable joints prone to sprain, dislocation, subluxation and hyperextension
- Early onset of advanced osteoarthritis
- Chronic degenerative joint disease
- Swan neck deformity of the fingers
- Weak muscle tone in infancy, which can delay the development of gross motor skills such as sitting, standing and walking
- Osteopenia (low bone density)
- Stretchy ligaments and tendons
- Tearing of tendons or muscles
- Deformities of the spine, such as: Scoliosis (curvature of the spine), Kyphosis ( thoracic hump), tethered spinal cord syndrome
- Myalgia (muscle pain) and arthralgia (joint pain)
There are other symptom lists relative to other EDS classifications, but I will only list those that are specific, common symptoms also experienced by many diagnosed with FM.
Skin
- Stretchy skin
Cardiovascular
- Valvular heart disease (mitral valve prolapse)
- Postural orthostatic tachycardia syndrome (POTS) – low blood pressure
- Orthostatic intolerance – upright posture causes blood pooling in lower body, leading to a 44-60% decrease in cerebral blood flow velocity
- Vascular skin conditions such as Raynaud’s phenomenon
Other related symptoms
- Digestive disorders such as gastritis, gastroesophageal reflux disease, irritable bowel syndrome,
(Other related symptoms cont)
- diverticulitis, gastroparesis (partial paralysis of the stomach)
- Nerve compression disorders such as carpal tunnel syndrome
- Anxiety disorders, depression, psychosocial dysfunction
- Insensitivity to local anesthetics
- Arnold-Chiari malformation
- Cranial instability
- Migraines
- Problems with concentration and/or memory
- Temporomandibular join syndrome
- Surgical complications due to tissue fragility
The important relevant information we heard in Dr. Graham’s talk is that EDS might be yet another example of a lesser known medical illness being diagnosed as fibromyalgia. If you notice that you experience many of these symptoms, most importantly joint hypermobility, it might be a good idea to ask your doctor about the possibility of being tested for EDS. At the very least this suggestion might open the doors to more effective treatment for your many FM symptoms.
Visit the Ehlers-Danlos Foundation site http://www.ednf.org/
