Hypermobility increases risk of osteoporosis

Patients with hypermobility have a significant and often unappreciated risk for osteoporosis. The prevalence of osteopenia and osteoporosis is very high in this cohort.

In patient population, this is something that we're aware of and tend to screen for osteoporosis sooner rather than later, particularly if there are any other risk factors.

Additional complications of hypermobility include problems with proprioception, osteoarthritis, mitral valve prolapse, hernias, passing out, palpitations, chest pain, fatigue, and heat intolerance.

These patients don’t just complain of pain in their joints; they complain of lots of things. They maybe have headaches and chronic pain. Sometimes pain disrupts their sleep and they get secondary fibromyalgia. [They could have] problems with sexual relations. They may have injuries and [psychological] reactions to the injuries. They have resistance to local anesthetics.

One of the reasons why hypermobility can be difficult to diagnose is that patients may not always have pain in the affected joints. If a patient has pain, they protect their joint and develop arthritis in the joint. Reducing range of motion and disguising the hypermobility.

One specific type of hypermobile disease is Ehlers-Danlos syndrome, which as 10 stubtypes, all characterized by slightly different associated comorbidities and risk factors. One of the most serious of these (type 4) may be fatal, but is also especially rare, with an estimated prevalence of 1 in 250,000. Hallmark signs are vascular aneurysms, bowel and organ rupture, milder hyperextensibility compared with other types, translucent skin, pinched nose, dystrophic scars, and severe ecchymosis, which can often lead doctors to think that the sufferer is being physically abused.

So how do we manage these people? We mentioned that screening for mitral valve prolapse — both by listening and, if indicated, echocardiogram — is very important. We discourage the hypermobile activities of daily living, although in the musician and the dancer we have to put practicality together — and make compromises.

Additionally, physical therapy can help these patients, as can measures to protect fragile skin. Recurrent dislocations can be treated with surgery, but sutures must be placed very carefully and closely, and with “careful hemostastis.”

All [of the patients] who have severe disease are in need of genetic counseling as well.

Another serious hypermobility disease in Marfan syndrome. The criteria for a Marfan diagnosis is complicated, but that forma practical standpoint, if a patient’s arm span measures more than 1.1 times his height, that is a good sign that Marfan syndrome may be present.

These patients can suffer complications like scoliosis, pectus excavatum, and pectus carinatum, also known as “pigeon chest.” That can be so severe that it actually can compress the heart. These patients also often have a high, arched palate.

In Marfan syndrome, Ehlers-Danlos syndrome, and all hypermobility disorders emphasized that “management is multidisciplinary. People really have to work together as a team. The patient, the rheumatologist, the orthopedist, and the primary care providers all need to work together with the physical and occupational care therapist in dealing with these diseases.

Diagnosis criteria for hypermobility

A diagnosis can be made if a patient has both of the major criteria, or if the patient fulfills one major criteria and two minor criteria, or simply has four minor criteria. The Beighton hypermobility score assigns up to 9 points based on a patient’s ability to bend and flex certain parts of the body.

Major criteria

• A Beighton score of greater than 4 (out of 9).

• Arthalgias for longer than 3 months in more than four joints.

Minor criteria

• A Beighton score of 1-3

• Arthralgia in one to three joints.

• History of joint dislocation.

• More than three soft tissue lesions.

• Marfanoid habitus (tall and slim, with a span: height ratio greater than 1.03, and an upper lower segment ratio less than 0.89).

• Skin striae, hyperextensibility, or scarring.

• Lid laxity

• History of varicosity, hernia, visceral prolapse.