Joint hypermobility syndrome is a disease in which excessive mobility is observed, combined with pathological symptoms from the musculoskeletal system. Usually patients are concerned about joint and muscle pain. Synovitis, bursitis, enthesopathy, repeated subluxations, early arthrosis, and other manifestations are possible. The diagnosis is made on the basis of anamnesis, clinical examination data. To exclude other diseases, laboratory and instrumental studies are prescribed. Treatment includes drug therapy, physical therapy, correction of the daily routine and motor activity.
M35.7 Hypermobile syndrome of looseness, excessive mobility
Joint hypermobility syndrome (JHS) is a pathological condition that should be distinguished from asymptomatic joint hypermobility (JH), which is not accompanied by clinical manifestations. The prevalence of JHS in the population is about 4%. Women get sick more often than men (5.6% and 1.9%, respectively). 65% of patients have close relatives suffering from the same disease, which indicates the hereditary nature of the pathology. JHS is associated with an increased risk of osteoarthritis and other diseases of the musculoskeletal system.
Generalized hypermobility of the joints is a widespread condition. According to researchers, an increase in the volume of movements compared to the average norm is detected in 6.9-31.5% of the inhabitants of the planet, more often – in women, natives of Asia, Africa, the Middle East. Excessive mobility is based on mutations of genes responsible for the formation of collagen and tenascin X, which are part of ligaments, tendons and joint capsules.
This individual feature is inherited, mainly through the female line. This disorder – connective tissue dysplasia (CTD) is potentiated by the influence of many genes, while a unique combination of alleles of both parents is important, which explains the significant variability in the manifestations of JH, the uncertain probability of the development of JHS, as well as the presence or absence of symptoms of connective tissue insufficiency from other organs and systems.
A change in the structure of connective tissue leads to a decrease in its strength and, as a consequence, to an increase in the likelihood of microtrauma during physical exertion. Along with repeated microtrauma, it is important to reduce the threshold of pain sensitivity and the presence of orthopedic anomalies that are often detected in patients with CTD – flat feet, hip dysplasia, spondylolisthesis, scoliosis.
A certain role is played by vegetative-vascular disorders, which also often accompany CTD. All of the above causes the appearance of pain in the muscles and joints against the background of physical activity. With age, the manifestations of JHS are aggravated, because acquired pathological changes are added to congenital anomalies – meniscopathy, enthesopathy, tendinitis, osteochondrosis.
The first symptoms usually appear at a young age. Patients complain of muscle and joint pain after moderate physical activity or minor injury. As a rule, the greatest intensity of pain is noted in the ankle and knee joints. There are no signs of inflammation. The recurrent nature of pain forces patients to limit motor activity, which leads to detrenirovannosti, increased likelihood of injury during physical exertion.
Against the background of detrenirovannosti, muscle weakness, increased fatigue appear. Sometimes acute synovitis develops after injuries, typical features of which are the non-inflammatory nature of effusion and rapid relief of symptoms. Back pain is often a concern. The intensity of the pain syndrome can vary significantly – from minor or moderate pain to persistent pain, significantly limiting the physical capabilities of the patient.
Since JHS develops against the background of congenital disorders of the synthesis of proteins that make up the skin, vascular walls, and internal organs, many patients have extra-articular manifestations of pathology. Typical signs are excessively stretched thin skin, early development of varicose veins, bruising after minor injuries. In some patients, mitral valve prolapse, prolapse of the uterus, kidneys, rectum, hernial protrusion of various localizations are detected.
In patients with joint hypermobility syndrome, repeated subluxations of the joints may occur. Over time, the pain begins to bother even in the absence of significant loads, which is associated with the formation of meniscopathies, enthesopathies, bursitis, tunnel syndromes, and other pathologies. There is an increased likelihood of early osteoarthritis and osteochondrosis. With the manifestation of secondary lesions of the ligamentous-articular structures, complaints are modified, correspond to the clinical manifestations of a particular disease.
The presence of vegetative disorders, often detected in connective tissue dysplasia, aggravates the course of the disease. Against the background of constant arthralgias, myalgias and dorsalgias, combined with fainting, cardialgia, palpitations, a feeling of lack of air, and other signs of autonomic dysfunction, neurotic disorders develop.
Joint hypermobility syndrome is diagnosed by orthopedists or rheumatologists. To detect increased mobility, the so–called Bayton score is used – a set of signs that are detected during simple tests that do not require additional equipment:
- Overextension of the little finger. The straightened little finger is deflected to the back of the hand until an angle of 90 degrees is formed.
- Bringing the I finger. By holding the thumb with the other hand, it can be brought close to the forearm.
- Overextension of the elbow joint. The forearm deviates towards the shoulder up to 10 degrees or more.
- Overextension of the knee joint. The angle between the thigh and the shin is 10 degrees or more.
- Increased flexibility of the spine. In the standing position, the patient can completely put his palms on the floor without bending his knees.
For the first four positive signs, 1 point is awarded on each side, for the fifth – 1 point. The highest possible number of points is 9. Using the test results and anamnesis data, JHS is detected based on certain diagnostic criteria:
1. Big criteria:
- 4 or more points on the Beighton scale;
- complaints of pain in 4 or more joints for 3 or more months.
2. Small criteria:
- 1-3 points on the Bayton score for young patients and 0-2 points for people over 50 years old;
- arthralgia in 1-3 joints or lumbalgia for 3 or more months, spondylosis, spondylolisthesis;
- a history of dislocations in more than 1 joint or repeated dislocations of the joint;
- lesion of the periarticular tissues: bursitis, tendinitis, enthesopathy, epicondylitis, etc.;
- tall stature, thinness, arachnodactyly, long legs;
- skin changes: atrophy, excessive extensibility, striae, etc.;
- myopia or overhanging eyelids;
- varicose veins of the lower extremities, omission of internal organs, hernias.
To make a diagnosis, you need 2 large signs, 1 large and 2 small signs, or 4 small signs. The examination plan for suspected JHS is determined by the complaints of patients, may include radiography, CT, MRI of various segments, and other imaging techniques. To exclude rheumatic diseases, appropriate laboratory tests are performed.
Hypermobility of the joints, combined with symptoms from articular, periarticular and muscle tissues, occurs in a number of other diseases, which determines the importance of differential diagnosis. First of all, JHS is distinguished with Marfan and Ehlers-Danlos syndromes. In some cases, differentiation with Louis-Dietz syndrome, osteogenesis imperfecta, and other pathologies may be required.
Treatment of joint hypermobility syndrome is long-term, carried out on an outpatient basis. The central place in the plan of therapeutic measures is occupied by the correction of lifestyle and physical activity regime. According to indications, non-drug methods of treatment are supplemented with drug therapy.
When using physiological methods to normalize the condition of patients, an integrated approach and early initiation of treatment are important. Of particular importance are non-drug methods in the treatment of patients under 20 years of age, in whom it is often possible to significantly reduce the manifestations of JHS during later life. Recommended:
- Optimal daily routine. It includes a sufficient amount of sleep, alternating periods of work and rest, regular full meals. An important part of the regime is daily moderate physical activity to prevent detrenirovannosti.
- Correction of motor stereotypes. Provides for working out the correct technique of movements. It is carried out by physical therapy doctors. It is aimed at eliminating non-physiological loads, preventing overloads, repeated traumatization.
- Kinesiotherapy. Complexes of static and dynamic exercises are being developed to strengthen muscles in problem areas. Physical therapy complexes are performed first with a specialist, then individually at home, according to indications, supplemented with osteopathy, myofascial releasing, mechanotherapy.
- Fixation. Individual selection of orthoses is made. During the selection, the level of activity of the patient, the localization of the pain syndrome, the severity of changes in various anatomical zones are taken into account.
- Physical therapy. It is prescribed during exacerbations. It is aimed at eliminating pain syndrome, stimulating tissue repair. Medicinal electrophoresis, laser therapy, magnetotherapy, and other techniques are used.
In most cases, non-drug measures in combination with the use of local remedies (gels, ointments) with distracting and anti-inflammatory effects are sufficient to eliminate the pain syndrome. With insufficient effectiveness, the following are shown:
- Stimulants of protein metabolism. The list of recommended products includes calcitonin, ascorbic acid, nicotinic acid, B vitamins, mineral complexes, and other drugs that promote the activation of collagen formation, ensuring the balance of redox processes.
- Correctors of glucosaminoglycan metabolism. Medicines from the group of chondroprotectors are used – glucosamine sulfate, chondroitin sulfate, etc.
- Stabilizers of mineral metabolism. Products containing various forms of vitamin D (ergocalciferol, alfacalcidol and analogues), calcium preparations are used.
- Correctors of the bioenergetic state. Medications are prescribed, which include phospholipids and polyunsaturated fatty acids (lecithin), riboxin, meldonium, essential amino acids.
Drug therapy is carried out several times a year in courses lasting about 2 months with an interval between courses of at least 2-3 months. Usually the course includes one drug from each group. At the beginning of the next course, the funds are replaced. Medication is alternated with physical therapy. NSAIDs for joint hypermobility syndrome are not recommended because of the low severity of inflammation and the possible negative effect of medications on the condition of connective tissue.
The prognosis is favorable. With the observance of the recommended activity regime, the development of correct motor stereotypes, most patients lead a normal lifestyle, fully retain their ability to work. In some cases, a severe course with repeated episodes of disability, the need for individual adaptation, and sometimes a forced change of profession is possible.
Primary prevention has not been developed due to the congenital nature of the pathology. Secondary preventive measures include early detection and regular monitoring of patients with joint hypermobility, individual selection of physical activity, career guidance, explanation of the features of the course of the disease to prevent neurotic disorders, creating a mood for the need for correction throughout life.
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