Benign Hypermobility joint syndrome (BHJS) is also called constitutional hypermobility or joint laxity. The disease is associated with an increased range of motion in the joints resulting from abnormalities in the structure of connective tissue. What are the causes and symptoms of joint laxity? How is the treatment going?

Benign Hypermobility joint syndrome - BHJS ,constitutional hypermobility ,joint laxity ) is a disease whose prevalence varies geographically. The disorder affects up to about 38 percent of the Asian and African population, while in Western countries the problem affects about 10% of the population. Women get sick three times more often than men.

In addition, most cases concern children and adolescents, because their connective tissue is not fully developed and in the elderly, symptoms may be masked by the aging process. A positive family history also increases the likelihood of BHJS, this is especially pronounced in twins. So far, no epidemiological studies have been conducted in Poland, because the inconsistent criteria for assessing this syndrome make it difficult to keep accurate statistics. However, among selected groups of patients the incidence rate was comparable to that presented in the international literature.

Joint laxity: causes

BHJS is usually associated with one of the following pathologies:

  • incorrect formation of one or more bones forming a joint;
  • defect of collagen or other tissues (e.g. associated with the occurrence of genetic diseases such as Ehlers-Danlos, Marfan, Loeys-Dietz syndrome); this results in the weakening of the elements that build joints: ligaments, muscles or tendons;
  • abnormal proprioceptive sensation, which correctly determines the ability to feel how much a joint can be stretched;

These abnormalities lead to excessive strain on the joints, excessive wear of their components and ultimately to osteoarthritis. It should be remembered that we may be dealing with secondary hypermobility of the joints, which is the result of too intensive training at a young age. Children who practice ballet and acrobatics, for example, are particularly vulnerable.

Joint laxity: types

1.Pathological local hypermobility- disorders concern one joint:

a. primary form - is a balancing element that compensates for the "stiffening" of the adjacent joints,

b. secondary form - resulting from complications resulting from damage to a single joint.

2.Generalized pathological hypermobility- associated with congenital disorders in the structure of connective tissue.

3.Constitutional hypermobility (HC)that is mild joint hypermobility; excessive elasticity results from a disturbance of the proportion of different types of collagen in the connective tissue.

Joint laxity: symptoms

  • chronic joint pain is the most common symptom; it can affect any joint, but mainly manifests itself in the knee and ankle joints (it is related to the high load on these joints when standing and walking); excessive physical activity exacerbates pain; in children, the pain is often increasing, it intensifies in the evening and at night, it can also wake up from sleep;
  • back pain, increased muscle tension in the paraspinal muscles;
  • subluxations or dislocations of the joints can occur as a result of injury, but also during normal physical activity; however, they do not apply to all patients;
  • may coexist: flat feet, valgus knees, curvature of the spine, enlarged lordosis of the lumbar spine, varicose veins, thin skin.

Joint laxity: diagnostic criteria

The modified Beighton scale is used to evaluate excessive joint mobility. It is assessed whether the patient is able to perform:

  • elbow hyperextension>10 °,
  • passive thumb to forearm,
  • hyperextension in MCP joints>90 °,
  • knee hyperextension>10 °,
  • put your hand flat on the floor while bending forward with your knees extended.

Positive performance of each activity is scored (1 point for each limb - the first four points and 1 point for the last). A score of 4 or more out of 9 indicates excessive joint mobility.Special criteria called Brighton criteria have been created to diagnose BHJS:

1. Major criteria:

a. fulfilled ≥ 4 of 9 points on the Beighton scale,

b. joint pain in ≥ 4 joints lasting more than 3 months

2. Minor criteria:

a. 1 to 3 points on the Beighton scale met;

b. persistent pain 1-3 joints or in the lumbosacral area>3months, degenerative changes in the spine or spondylolisthesis;

c. displacement or subluxation within>1 joint or multiple within one joint;

d. soreness within the periarticular tissues;

e. structure similar to that observed in Marfan syndrome;

f. changes in the skin (stretch marks, excessive stretch, "parchment" scars);

g. drooping eyelids, myopia, eye skew (opposite to Mongolism);

h. varicose veins, hernias, uterine prolapse or rectal prolapse.

To diagnose BHJS, the following must be stated:

  • two larger criteria,
  • one major and two smaller criteria,
  • four minor criteria,
  • two smaller criteria, where the patient is a first degree relative of a person diagnosed with BHJS.

Always perform: a complete blood test, ESR with tests for detection of rheumatoid factor, serum antinuclear antibodies, immunoglobulins (IgG, IgM, IgA) to rule out inflammatory or immunological causes of symptoms. In addition, due to the possible coexistence of serious diseases, such as:

  • Down syndrome
  • Ehlers-Danlos syndrome
  • Marfan's syndrome
  • Marquio band
  • Loeys-Dietz syndrome
  • clavicle-cranial dysostosis (inherited bone developmental disease)
  • Stickler's team
  • congenital fragility of bones
  • there are studies suggesting an association of BHJS with carpal tunnel syndrome
  • fibromyalgia

Detailed diagnostics is recommended to rule them out.

Joint laxity: treatment

Due to the causes of the disease, there is no causal treatment. The basis of therapy is appropriately selected rehabilitation. It is advisable to strengthen muscle strength and proprioception in the area of ​​the joint affected by hypermobility. Remember that stretching exercises are inadvisable, instead exercises improving the stabilization of the joints are recommended.

We encourage patients to take daily physical activity, because the consequence of inactivity is muscle weakness, which in turn promotes the progression of movement disorders. Swimming and cycling are the best choices for relieving the joints. In addition to rehabilitation, warm or cold compresses, TENS treatment, massages and various forms of relaxation are helpful in the treatment of pain.

Category:

Spine, bones, joints