Benign Joint Hypermobility Syndrome

If you have benign joint hypermobility syndrome (BJHS), also more simply known as “double joints” or “loose joints”, you have probably been very flexible in some or all of your joints since you were a child. However, benign joint hypermobility syndrome goes well beyond just being able to bend, hyperextend or move beyond a typical range of motion. This syndrome causes mild to severe pain in the joints plus an increasing risk of osteoarthritis and joint injury.

People that are double jointed or have an increased range of motion without any pain do not have benign joint hypermobility syndrome, and this is an important distinction to make.  In fact it is estimated that between 4 to 13% of the population have hypermobility of the joints that is not related to any systemic disease or condition. Women are more likely to have joint laxity than men, particularly in research studies completed with large numbers of high school age participants. 1

There are some other syndromes that also have hypermobility of the joints as one of the key symptoms. These include Marfan syndrome and Ehlers-Danlos syndrome. Marfan syndrome is a genetic connective tissue disorder that causes increased growth of the limbs and skeleton, eye problems, serious complications with the central nervous system, cardiovascular system, respiratory system and the eyes. This condition is typically diagnosed early in a child’s life and is usually found within specific families and considered rare in the general population.

Ehlers-Danlos syndrome is a dysfunction in the ability of the body to synthesize collagen. This results in a poor connective tissue function which leads to structural problems with the skin, blood vessels, muscles and the connective tissues in the body, including the supportive tissue for internal organs. Extreme flexibility of the joints, including the ability to bend joints backwards to their maximum range is often seen in this condition. Like Marfan syndrome, Ehlers-Danlos syndrome is inherited, is very rare in the general population, and is not curable but can be managed in most individuals.

Symptoms of Benign Joint Hypermobility Syndrome   

Since this condition typically begins to occur in children and teens there is a link between the joint and muscle pain and “growing pains”. Often the more significant benign joint hypermobility syndrome is seen as a natural part of development, which may limit the options for management of the pain in teens and older children. However the symptoms of benign joint hypermobility syndrome may include slight or mild swelling of the affected joints, particularly after exercise or use. This is often more pronounced during the late afternoon and evening and seems mild or non-existent in the morning.

There are no other visible signs, and redness, extreme or moderate swelling or heat in the affected joints on the surface of the skin is not present. There are also no associated symptoms. Growth disorders or genetic syndromes or other health issues may have the same joint pain and hypermobility as a symptom. Of course the child, teen or adult also has a higher than normal range of motion in the affected joint. This can lead to increased risk of sprains, strains and joint trauma, but this is not a symptom of the condition, rather it is a result of the hypermobility of the joint itself. 2

General symptoms that medical experts look for in the diagnosis of benign joint hypermobility syndrome include:

  • Thumb can be bent down to touch the forearm
  • Knees bow back more than 10 degrees when the individual is standing
  • Little finger can bend back 90 degrees
  • Arms can be hyper-straightened at the elbow to bend backwards
  • Palms can be placed flat on the ground when bending at the waist with the knees straight.

In addition to these general tests there is also a specific rating test known as the Brighton criteria that can be used by medical professionals. This rates the looseness of the joints on a scale with a score of 4 or more considered indicative of the syndrome. Other researchers have worked to develop a patient self-reporting questionnaire that is simple, easy to answer and highly accurate. This self-reporting questionnaire will help in distinguishing benign joint hypermobility syndrome from other types of chronic pain syndromes. 3

Treatment of BJHS

The specific treatment of benign joint hypermobility syndrome tends to focus in on management of symptoms, controlling the pain and strengthening the muscles and joints. Exercise is often done in water to limit the damage of possible strains, sprains and ligament or tendon damage. Warm water exercise has also been effective in treatment of overall fibromyalgia symptoms, making this a good option to consider.

Controlling motions and learning to avoid hyperextension of joints may also help in pain management and decrease the risk of any further damage to the joints. Posture, both seated and standing, can help in decreasing lower back and neck pain if hypermobility in the spine is an issue.

Anti-inflammatory medications such as ibuprofen or naproxen can be helpful to relieve swelling if it occurs due to muscle or joint injury, but the pain from benign joint hypermobility syndrome is not due to inflammation. In this case these medications will not be effective and will not help to decrease the pain experienced. Since benign joint hypermobility syndrome is associated with higher risk for developing arthritis, early preventative and supportive treatment for joint health may worth discussing with a rheumatologist or your treating physician.

Since benign joint hypermobility syndrome, fibromyalgia, chronic fatigue syndrome and other chronic pain syndrome are often experienced together, researchers now believe that a genetic factor, such as collagen processing enzymes or specific glycoproteins, may be the cause of the overlapping symptoms.  One promising research study links a deficiency in tenascin-X, a large glycoprotein, as a contributing factor in benign joint hypermobility syndrome. 4

A better understanding of the underlying factors that cause the development of benign joint hypermobility syndrome and fibromyalgia will help in developing a more effective treatment. Research into determining why some children and adults have joint laxity but not benign joint hypermobility syndrome will also be critical to understanding this common yet complicated medical condition and its role in patients with fibromyalgia and chronic fatigue syndrome.

References

1 Seckin, U., Tur, B., Yilmaz, O., et al. (2005). The prevalence of joint hypermobility among high school students. Rheumatology International , 260-263.

2 Simpson, M. R. (2006). Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. Journal of the American Osteopathic Association , 531-536.

3 Hakim, A., & Grahame, R. (2003). A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain. International Journal of Clinical Practice , 163-166.

4 Zweers, M. C., Hakim, A. J., Grahame, R., et al. (2004). Joint hypermobility syndromes: The pathophysiologic role of tenascin-X gene defects. Arthritis & Rheumatism , 2742-2749

This article was originally published on July 11, 2012 and last revision and update of it was 9/7/2015