An Overview of Joint Hypermobility Syndrome

Loose joints lead to pain and potential injury

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Joint hypermobility syndrome, sometimes called benign hypermobility joint syndrome (BHJS), is an inherited connective tissue disorder. It is associated with three classic findings: generalized hypermobility (characterized by greater than average range of mobility in the joints), chronic joint pain, and other neuromuscular signs due to a defect in collagen. Importantly, this condition occurs without signs of inflammation and it may be necessary to rule out a more systemic disease affecting the body with the help of testing or evaluation by a rheumatologist.

Symptoms

Clearly, given the name, symptoms of joint hypermobility syndrome most often directly affect the joints of the body, often the larger joints such as the knees or elbows. Weight-bearing joints, usually in the legs, may be more obviously impacted. In fact, any joint may be involved, including the hands, shoulder, patella (kneecap), and even the jaw joint (temporomandibular joint or TMJ).

One joint, or multiple, may be involved. The findings may be symmetric, affecting both sides of the body fairly equally, or more generalized.

Symptoms may be self-limited, varying over hours, and may resolve without intervention. Alternatively, they may become constant and affect someone chronically.

The most common symptoms of joint hypermobility syndrome include:

  • Joint pain (often worsened late in the day)
  • Morning stiffness
  • Mild joint swelling (noted in the late afternoon or at night or after intense exercise or repetitive activity)
  • Less commonly muscle cramps
  • Easy bruising
  • Recurrent dislocations (typically at the shoulder or patella)
  • Ligament or tendon ruptures
  • Fatigue
  • Anxiety or panic disorder

There are some findings that would actually lead away from a diagnosis of joint hypermobility syndrome and may instead suggest another inflammatory disorder such as rheumatoid arthritis. These symptoms may include fever and redness or warmth affecting joints.

Causes

Joint hypermobility syndrome is a surprisingly common condition. Generalized joint laxity alone is common in healthy individuals without other complaints. This hypermobility, which ultimately is not associated with other systemic diseases, occurs in between 4% and 13% of the population.

Joint hypermobility syndrome with its additional findings often runs in families due to a genetic predisposition.

With an autosomal dominant pattern of inheritance, the syndrome is easily passed through families. It is estimated that 50% of people with joint hypermobility syndrome have another close family member who is affected (such as parents, siblings, or children).

The predisposition towards the condition may relate to several genetic mutations affecting:

  • Collagen
  • The ratio of collagen subtypes
  • Fibrillin

As the predisposition for the condition may be present from birth, it is estimated that joint hypermobility syndrome may impact up to 40% of schoolchildren and 10% may have associated pain complaints after activities or at night. These children may demonstrate extreme flexibility, making them more successful at gymnastics, cheerleading, ballet or dance, and yoga.

The prevalence may diminish with aging, as the looseness of joints becomes less apparent. It may occur more among girls and women. Hypermobile joints may cause symptoms in 5% of healthy women but in only 0.5% of men.

Finally, certain ethnicities seem to have a higher incidence of joint hypermobility syndrome. It more commonly affects people of Asian descent compared to those who come from Caucasian or African descent.

Diagnosis

The diagnosis of joint hypermobility syndrome may be accomplished with a few questions, a brief physical examination to assess potential signs, and a handful of blood tests to rule out other potential causes. It may be important to differentiate similar syndromes that may have overlapping symptoms.

Questions Your Doctor Will Ask

Most people may seek initial evaluation by their primary care provider before seeing a specialist, such as a rheumatologist or a physiatrist. A few questions may suggest the possibility of needing to take this first step in assessment, including:

  • Are you double-jointed?
  • As a child or teen, did your shoulder or kneecap dislocate on more than one occasion?
  • Did you ever amuse your friends by contorting your body into strange shapes or could you do the splits?

Physical Examination

The hypermobility and pain affecting multiple joints that characterize joint hypermobility syndrome may be assessed with the Brighton criteria to establish the Beighton score (similar names that describe slightly different things).

When joint pain (arthralgia) is present for three months or longer and joint laxity affects four or more joints, the condition is diagnosed.

This assessment can be done by a physician who observes five simple maneuvers in an assessment that takes less than 60 seconds. Greater range of motion in joints is measured by these specific mobility tests:

  • Wrist and thumb can be moved downward so the thumb touches the forearm (bend thumb to touch forearm)
  • Little fingers can be extended back beyond 90 degrees
  • When standing, the knees are abnormally bowed backward when viewed from the side
  • When fully extended, the arms bend further than normal (beyond straight)
  • When bending at the waist, with the knees straight, can put palms flat on the floor (place your hands flat on the floor without bending your knees)

Beyond these physical examination maneuvers, it may be necessary to do some blood tests to rule out other potential causes of the symptoms.

Blood Tests

A careful history and physical examination may be enough to identify the symptoms and signs of joint hypermobility syndrome. When the diagnosis is unclear, or when overlapping symptoms are present, it may be necessary to rule out other inflammatory, infectious, and autoimmune causes.

The workup may include some additional blood tests, such as:

  • Complete blood cell count
  • Erythrocyte sedimentation rate (ESR)
  • Rheumatoid factor
  • Antinuclear antibody test
  • Serum complement levels
  • Serum immunoglobulin (IgG, IgM, and IgA) levels

This need for this testing should be directed by your physician.

Signs

The underlying predisposition for laxity in joints and connective tissues may lead to other problems that affect the body. Wear and tear on joint surfaces may lead to an increased predisposition for strains, dislocations, and tears. Impairment in the ability to sense body position in space (called proprioception) may lead to a higher incidence of joint trauma due to impaired sensory feedback.

Consider this expansive list of potential signs and associated conditions that occur more often in the context of joint hypermobility syndrome:

  • Sprains
  • Tendon or meniscus tears (affecting the knee)
  • Dislocations (affecting the shoulder, patella, or TMJ)
  • Fractures
  • Tendonitis
  • TMJ dysfunction
  • Rotator cuff syndrome
  • Back pain
  • Scoliosis
  • Lordosis
  • Nerve compression disorders (carpal tunnel syndrome)
  • Congenital hip dysplasia
  • Bowleggedness (knock knee or genu valgum)
  • Flat feet (pes planus)
  • Chondromalacia
  • Unspecified arthralgia or effusion
  • Osteoarthritis
  • Fibromyalgia
  • Obstructive sleep apnea
  • Autonomic dysfunction (lightheadedness, syncope, etc.)
  • Raynaud syndrome
  • Bruising
  • Varicose veins
  • Skin striae (thin skin, hyperextensibility, scarring)
  • Ocular signs of drooping eyelids, myopia
  • Mitral valve collapse (controversial)
  • Hernia
  • Uterine or rectal prolapse

Beyond these findings, there are similar conditions that may share some symptoms and should be ruled out based on distinguishing characteristics.

Differential Diagnosis

There are some symptoms and signs of joint hypermobility syndrome that may overlap with other disorders.

  • Marfan syndrome: An inherited disorder that affects connective tissue and may cause an affected person to be double-jointed with disproportionately long arms and legs, a tall and slender build, arachnodactyly (elongated fingers), and cardiac and ocular features (such as myopia and lens dislocation)
  • Ehlers-Danlos syndrome: An inherited disorder that mostly affects skin, joints, and blood vessels (leading to symptoms such as being double-jointed, bleeding, and muscle pain)
  • Osteogenesis imperfecta: Characterized by a defect in collagen, this genetic disorder leads to excessive joint laxity, thin blue sclera, and bone fragility that leads to multiple fractures and bony deformities.
  • Down syndrome: A genetic disorder caused by trisomy of chromosome 21 that leads to characteristic physical findings, including a distinct facial appearance, developmental delay or intellectual disability, and thyroid or heart abnormalities.
  • Metabolic disorders: Including homocystinuria and hyperlysinemia

When other conditions are suspected, further evaluation may be necessary to clarify the diagnosis. It may be necessary to have testing to rule out arthritis (especially juvenile arthritis in children), inflammatory conditions, or even X-rays to assess for fractures or other abnormalities.

Once the proper diagnosis is made, the proper treatment may be pursued.

Treatment

Generally, joint hypermobility syndrome is a chronic disorder with symptoms that may fluctuate over time.

Seek help if you experience a sudden change in the ability to move a joint or if a joint suddenly becomes misshapen, as this may require acute intervention.

For most people affected by joint hypermobility syndrome, the condition is experienced as a non-progressive, non-inflammatory condition that may be improved with simple lifestyle modifications.

  • Maintain good nutrition: Weight gain can negatively affect joint health, especially the knees, and taking a daily multivitamin may ensure that the right nutrients are available for tissue building and repair.
  • Stretching and strength-building exercises: Low-impact exercises may reduce injury risk and improve fitness. It may be especially important to target the strengthening of muscles around loose joints. By isolating these muscle groups, and improving balance and control, the stress of the joints may be reduced. Beyond improving joint stability and proprioception, optimized balance and coordination may help to avoid inadvertent injuries.
  • Joint protection: During certain activities, such as vigorous or repetitive exercise, it may be necessary to wear splints or braces or tape affected joints to protect them. Wear shoes or footwear with good arch supports. Supportive splints may be required for specific sports involvement.
  • Avoid certain positions and activities: It may be necessary to avoid vigorous and repetitive activities that strain the joints. Overtraining, poor pacing, and excessive performances or athletic competitions may lead to injury. Being conscientious about body postures and positioning may also be important. Bend the knees slightly when standing and avoid sitting cross-legged with both knees bent.
  • Know your limits: Try to avoid hypermobile joint movements that are likely to exacerbate pain and other symptoms.

Pain may be relieved with the use of non-steroidal anti-inflammatory drugs (such as ibuprofen or naproxen) or the use of acetaminophen. Rest and the avoidance of aggravating activities may be required.

In some cases, physical therapy may be necessary to recover from an injury. Osteopathic manipulative treatment (OMT) may increase joint motion, reduce pain, improve blood flow, aid lymphatic drainage, and enhance proprioception.

Coping

Joint hypermobility syndrome may be a chronic condition, but it does not have to be an overly limiting one. In fact, people who are affected may be more successful at activities that reward increased flexibility. This may lead to success in cheerleading, modern dance, gymnastics, and ballet.

Due to the increased risks for sprains, injuries, dislocations, occasional swelling, backaches, and discomfort after exercise, it is important to respect the body’s limits. It seems that after years of excessive joint motion, there is an increased risk for osteoarthritis (so-called “wear-and-tear” arthritis). This would require the same treatments, such as pain relief and surgery, that are often required with normal aging-related changes.

Poor sleep and symptoms of sleep apnea should prompt an evaluation as this may further undermine health and well-being.

A Word From Verywell

If you are concerned that you may be affected by joint hypermobility syndrome, seek evaluation by your primary health care provider. Supportive care, relief of chronic joint pain, and specific interventions may help you to feel more comfortable and to avoid potential injuries.

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Article Sources

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