JOINT HYPERMOBILITY SYNDROME in general practice

Is hypermobility always dangerous?

More ranged joint movements than necessary are typical mainly for children, and with age this problem gradually disappears. In such cases, treatment is not required and there are no complications. The reason is the development of joint tissue and the growth of cartilage. If the problem remains with a person into adulthood, this may indicate pathology - deformation of the joints and changes in the structure of blood vessels.

Plasticity and flexibility in childhood are sometimes off the charts! Below is a selection of the most flexible children in the world:

It's all about collagen

By the way, it is believed that this pathology occurs predominantly in female faces. Its prevalence is 5–7% of the total population, but every year the number of gutta-percha women and men is growing. This syndrome is more common among eastern peoples. There are especially many “rubber” people living in Asia, a little less of them in Africa and much less in Europe.

In some families, unusual stretch marks are inherited. But despite the fact that not in all cases people with snake-like flexibility need treatment, sometimes excessive stretchability of the ligaments can lead to problems.

The disease we are talking about today is a consequence of weakness of the ligamentous apparatus. And the reason for this phenomenon lies in molecular changes within the main structural protein of the body - collagen. The same protein is part of the skin, hair, nails, and it also makes up the walls of blood vessels and ligaments that support internal organs (it is due to these “suspensions” that the liver, kidneys, uterus and other organs can occupy the physiologically correct location in the body).

With a gross genetic mutation that disrupts the synthesis of collagen in the body, a person may experience severe, but, fortunately, rare hereditary diseases, in which patients experience excessive flexibility of the joints, and also in the first case, multiple bone fractures, and in the second - very tensile skin and the possibility of spontaneous rupture of blood vessels.

With joint hypermobility syndrome, the situation is less sad, but unpleasant consequences also exist. The altered protein impairs the firmness and elasticity of the ligamentous apparatus, as a result of which the ligament overstretches and becomes flaccid during the movement of the joint. Unfortunately, changes in the molecular structure of collagen in people with joint hypermobility syndrome are inherited and persist for life.

Since almost no system of the body can function without the participation of collagen, there can be many diseases associated with the weakness of connective tissue structures. This includes the early appearance of wrinkles, prolapse of heart valves (excessive sagging of the valves), prolapse of internal organs, and varicose veins. But most often, patients with joint hypermobility have problems with the joints and spine. In particular, this pathology can be considered a risk factor for the development of arthrosis.

Treatment tactics

As with the treatment of osteoarthritis, an integrated approach is required. Joints do not exist separately from other body systems, so it is necessary to influence from different sides. Most often, therapy includes the following methods:

• Taking painkillers.
• Physiotherapy aimed at strengthening ligaments and preventing their hyperextension (selected individually).
• Changing the patient's activity to reduce the intensity of pain.
• Psychological assistance is an explanation to a person that he does not suffer from a serious illness and does not risk becoming disabled.

Joint hypermobility is an unpleasant phenomenon, but often not dangerous.

Very rarely, hypermobility is a consequence of a serious illness. These include, for example, Ehlers-Danlos syndrome (“hyperelasticity of the skin”), Marfan syndrome (an autosomal dominant pathology of connective tissue of a hereditary nature).

Fortunately, in most cases, hypermobility is not potentially dangerous or disabling. Therefore, the main goal of therapy is to eliminate the pain syndrome and help the person psychologically accept this diagnosis. It is equally important to teach the patient to take care of himself in order to avoid injuries, since frequent injuries can provoke degenerative diseases in the joints, post-traumatic arthrosis and new problems.

JOINT HYPERMOBILITY SYNDROME in general practice

Joint hypermobility syndrome (JMS) is a common reason for patients of any age seeking medical help. The clinical manifestations of VHMS are varied and can mimic other, more well-known joint diseases. Due to insufficient familiarity with this pathology among general practitioners, and in some cases rheumatologists and orthopedists, the correct diagnosis is often not established. Traditionally, the doctor's attention is drawn to identifying limited range of motion in the affected joint, rather than determining excess range of motion. Moreover, the patient himself will never report excessive flexibility, since he has coexisted with it since childhood and, moreover, is often convinced that all people have the same capabilities. Two diagnostic extremes are typical: in one case, due to the absence of objective signs of pathology in the joints (except for visible hypermobility) and normal laboratory parameters in a young patient, “psychogenic rheumatism” is determined; in the other, the patient is diagnosed with rheumatoid arthritis or a disease from the group of seronegative spondyloarthritis and prescribe appropriate, by no means harmless, treatment.

What is considered joint hypermobility?

Among the many proposed methods for measuring range of motion in joints, Beighton's method, which is a nine-point scale assessing the subject's ability to perform five movements (four paired for the limbs and one for the trunk and hip joints), has received general recognition. Beighton proposed a simplified modification of the previously known method of Carter and Wilkinson (1964). The movements are shown in Fig. 1.

This is a simple and time-consuming screening procedure that has been widely used in both clinical and epidemiological studies. Based on a number of epidemiological studies, norms of joint mobility for healthy people were determined. The degree of joint mobility is distributed in a population in a normal way, that is, not according to the “all or nothing” principle, but in the form of a sinusoidal curve. The usual Beighton score for Europeans is from 0 to 4. But the average, “normal” degree of joint mobility varies significantly across age, gender and ethnic groups. In particular, when examining healthy individuals in Moscow aged 16-20 years, more than half of women, and more than a quarter of men, demonstrated a degree of HMS exceeding 4 points according to Beighton. Thus, in the absence of complaints from the musculoskeletal system, excessive joint mobility in comparison with the average can be considered as a constitutional feature and even an age norm. In this regard, in pediatric practice there are no generally accepted standards for joint mobility - this indicator changes significantly during the period of child growth.

Can excessive joint flexibility be acquired or is it a hereditary trait?

Acquired excessive joint mobility is observed in ballet dancers, athletes and musicians. Long-term repeated exercises lead to stretching of the ligaments and capsules of individual joints. In this case, local hypermobility of the joint(s) occurs. Although it is obvious that in the process of professional selection (dancing, sports) persons who are initially distinguished by constitutional flexibility have a clear advantage, the fitness factor undoubtedly takes place. Changes in joint flexibility are also observed in a number of pathological and physiological conditions (acromegaly, hyperparathyroidism, pregnancy). Generalized HMS is a characteristic feature of a number of hereditary connective tissue diseases, including Marfan syndrome, osteogenesis imperfecta, and Ehlers-Danlos syndrome. These are rare diseases. In practice, a doctor much more often has to deal with patients with isolated GMS, not associated with training and, in some cases, combined with other signs of weakness of connective tissue structures. It is almost always possible to establish the familial nature of the observed HMS and concomitant pathology, which indicates the genetic nature of the observed phenomenon.

Definition of joint hypermobility syndrome

The term “HMS syndrome” itself belongs to the English authors Kirk, Ansell and Bywaters, who in 1967 thus designated a condition in which there were certain complaints from the musculoskeletal system in hypermobile individuals in the absence of signs of any other rheumatic disease.

Subsequent work made it possible to specify the nosological boundaries of this syndrome, which was defined as “benign joint hypermobility syndrome,” in contrast to the prognostically more severe types of Ehlers-Danlos syndrome and other hereditary connective tissue dysplasias.

The latest, so-called Brighton criteria for benign HMS syndrome (1998) are presented in the table. These criteria also attach importance to extra-articular manifestations of weakness of connective tissue structures, which makes it possible to speak of HMS syndrome in persons with a normal range of motion in the joints (as a rule, we mean older persons).

Prevalence of joint hypermobility syndrome

HMS syndrome is understood as a combination of HMS and any complaints from the musculoskeletal system caused by weakness of the ligamentous system. The true prevalence of HMS syndrome is virtually unknown. Constitutional HMS is detected in 7-20% of the adult population. Although most patients first complain during adolescence, symptoms can appear at any age. Therefore, the definitions of “symptomatic” or “asymptomatic” HMS are quite arbitrary and reflect only the state of an individual with HMS at a certain period of life. We can talk about the frequency of detection of FHMS according to individual clinics. Thus, in one of the large European rheumatology clinics, this diagnosis was established in 0.63% of men and 3.25% of women out of 9275 patients admitted for inpatient examination. But these data do not reflect the true picture, since most patients with HMS syndrome do not require hospital treatment. According to domestic data, the proportion of patients with FHMS is 6.9% at an outpatient appointment with a rheumatologist (Gowert V.R., 1996). Due to the already mentioned insufficient knowledge of doctors about this pathology, these patients are often registered under other diagnoses (early osteoarthritis, periarticular lesions, etc.).

Clinical manifestations of HMS syndrome

The clinical picture of HMS is diverse and includes both articular and extra-articular manifestations, which are generally reflected in the mentioned Brighton criteria for HMS syndrome.

A careful history taking is a significant aid in diagnosis. A characteristic fact in the patient’s life history is his special sensitivity to physical stress and a tendency to frequent injuries (sprains, subluxations of joints in the past), which suggests a failure of the connective tissue. The excess range of motion in the joints detected by the Beighton method complements the actual clinical forms of manifestation of VHMS.

Articular manifestations

• Arthralgia and myalgia. The sensations may be painful, but are not accompanied by visible or palpable changes in the joints or muscles. The most common localization is the knee, ankle, and small joints of the hands. In children, severe pain in the hip joint has been described, responding to massage. The severity of pain is often influenced by the emotional state, weather, and the phase of the menstrual cycle.
• Acute post-traumatic articular or periarticular pathology, accompanied by synovitis, tenosynovitis or bursitis.
• Periarticular lesions (tendinitis, epicondylitis, other enthesopathies, bursitis, tunnel syndromes) occur more often in patients with VHMS than in the general population. They occur in response to an unusual (unusual) load or minimal trauma.
• Chronic mono- or polyarticular pain, in some cases accompanied by moderate synovitis provoked by physical activity. This manifestation of VHMS most often leads to diagnostic errors.
• Repeated dislocations and subluxations of joints. Typical locations are the shoulder, patellofemolar, and metacarpophalangeal joints. Sprained ligaments in the ankle joint.
• Development of early (premature) osteoarthritis. This can be either true nodular polyosteoarthrosis or secondary damage to large joints (knees, hips) that occurs against the background of concomitant orthopedic anomalies (flat feet, unrecognized hip dysplasia).
• Back pain. Thoracalgia and lumbodynia are common in the population, especially in women over 30 years of age, so it is difficult to make an unambiguous conclusion about the connection of these pains with joint hypermobility. However, spondylolisthesis is significantly associated with GMS.
• Symptomatic longitudinal, transverse or combined flatfoot and its complications: medial tenosynovitis in the ankle joint, valgus deformity and secondary arthrosis of the ankle joint (longitudinal flatfoot), posterior talar bursitis, thalalgia, corns, hammertoe deformity, Hallux valgus (transverse flatfoot) ).

Extra-articular manifestations. These signs are natural, since the main structural protein collagen, which is primarily involved in the described pathology, is also present in other supporting tissues (fascia, dermis, vascular wall).

• Excessive extensibility of the skin, its fragility and vulnerability. Striae not associated with pregnancy.
• Varicose veins that begin in youth.
• Mitral valve prolapse (before the introduction of echocardiography into widespread practice in the 70-80s, many patients with HMS syndrome were observed by a rheumatologist with a diagnosis of “rheumatism, minimal degree of activity” due to complaints of joint pain and heart murmurs associated with prolapse valves).
• Hernias of various localizations (umbilical, inguinal, white line of the abdomen, postoperative).
• Prolapse of internal organs - stomach, kidneys, uterus, rectum.

Thus, when examining a patient with suspected VHMS, and this is every young and middle-aged patient with non-inflammatory joint syndrome, it is necessary to pay attention to possible additional signs of systemic connective tissue dysplasia. Knowledge of the phenotypic manifestations of Marfan syndrome and osteogenesis imperfecta allows us to exclude these hereditary diseases. If obvious skin and vascular signs are detected (skin hyperelasticity and spontaneous formation of bruises without signs of coagulopathy), it is legitimate to talk about Ehlers-Danlos syndrome. The question of differential diagnosis of benign HMS syndrome and the “mildest”, hypermobile type of Ehlers-Danlos syndrome remains open. This cannot be done using the Brighton criteria, which the authors specifically mention; in both cases there is moderate involvement of the skin and blood vessels. There is no known biochemical marker for either syndrome. The question remains open and will, apparently, be resolved only with the discovery of a specific biochemical or genetic marker for the described conditions.

Considering the widespread prevalence of constitutional HMS in the population, especially among young people, it would be erroneous to explain all joint problems in this category of people only by hypermobility. The presence of HMS does not at all exclude the possibility of them developing any other rheumatic disease, to which they are susceptible with the same probability as persons with a normal range of motion in the joints.

Thus, the diagnosis of HMS syndrome becomes justified when other rheumatic diseases are excluded, and the existing symptoms correspond to the clinical signs of the syndrome, logically supplemented by the identification of excessive joint mobility and/or other markers of generalized connective tissue involvement.

Treatment of HMS syndrome

Treatment of a patient with HMS syndrome depends on the specific situation. The variety of manifestations of the syndrome also requires a differentiated approach to each individual patient. An important point is to explain in an accessible form the causes of his joint problems (“weak ligaments”) and convince the patient that he does not have a serious illness that threatens inevitable disability. For moderate arthralgia this is sufficient. Recommendations to eliminate stress that causes pain and discomfort in the joints will be useful. Decisive in the treatment of severe pain are non-drug methods, and first of all, optimizing lifestyle. This involves matching the loads and the threshold of their tolerance for a given patient. It is necessary to minimize the possibility of injury, which includes vocational guidance and the exclusion of team sports.

For persistent pain in one or more joints, elastic orthoses (knee pads, etc.) are used. Timely correction of detected flat feet is very important. At the same time, the doctor is required to have basic podological knowledge - the shape and rigidity of the insoles is determined individually, and the success of the treatment largely depends on this. It is often possible to cope with persistent arthralgias of the knee joints using this only method.

In ensuring the stability of the joint, not only the ligaments play a significant role, but also the muscles surrounding the joint. If it is impossible to influence the condition of the ligamentous apparatus through exercises, then strengthening and increasing muscle strength is a real task. Gymnastics for GMS syndrome has a peculiarity - it includes so-called “isometric” exercises, during which significant muscle tension occurs, but the range of motion in the joints is minimal. Depending on the location of the pain syndrome, it is recommended to strengthen the muscles of the thighs (knee joints), shoulder girdle, back, etc. Swimming is useful.

Drug therapy is applicable as symptomatic treatment for arthralgia. Since the pain associated with GMS syndrome is mainly non-inflammatory in nature, it is often possible to see a complete lack of effect from the use of non-steroidal anti-inflammatory drugs.

In this case, greater results can be achieved by taking analgesics (paracetamol, tramadol). Intra-articular administration of corticosteroids in the absence of signs of synovitis is absolutely ineffective.

For periarticular lesions (tendinitis, enthesopathies, bursitis, tunnel syndromes), treatment tactics are practically no different from those in ordinary patients. In moderate cases, these are ointments with non-steroidal anti-inflammatory drugs in the form of applications or compresses; in more persistent cases, local administration of small doses of glucocorticosteroids that do not have a local degenerative effect (suspension of methylprednisolone crystals, betamethasone). It should be noted that the effectiveness of local corticosteroid therapy largely depends on the correctness of the topical diagnosis and the technique of performing the procedure itself.

Note!

HMS is a common rheumatic syndrome that is not prognostically dangerous, but causes serious diagnostic problems in practice. A patient with suspected HMS syndrome requires the physician to pay attention to subtle details during history taking and examination; knowledge and experience are required in the ability to determine to what extent the nature of the complaints corresponds to the unusual joint mobility being detected. Treatment of GMS syndrome also has its own specifics and differs from traditional therapy for other patients with joint diseases

What to tell the patient?

It is important to explain to the patient in an accessible form the cause of his joint problems (“weak ligaments”) and convince him that he does not have a serious illness that threatens inevitable disability. For moderate arthralgia this is sufficient. Recommendations to eliminate stress that causes pain and discomfort in the joints will be useful; Advise on minimizing the potential for injury, which includes appropriate vocational guidance and avoidance of team sports.

Criteria for joint hypermobility syndrome

Large criteria

• Beighton score of 4 or more (at the time of examination or in the past)
• Arthralgia for more than 3 months in four or more joints

Small criteria

• Beighton score 1-3 (for people over 50 years old)
• Arthralgia for less than 3 months in one to three joints or lumbodynia, spondylosis, spondylolysis, spondylolisthesis
• Dislocation/subluxation in more than one joint or repeated in one joint
• Periarticular lesions of more than two localizations (epicondylitis, tenosynovitis, bursitis)
• Marfanoid (tall, thin, arm span/height ratio > 1.03, upper/lower body segment ratio < 0.83, arachnodactyly)
• Abnormal skin: thinness, hyperextensibility, stretch marks, atrophic scars
• Eye signs: drooping eyelids or myopia or antimongoloid fold
• Varicose veins or hernias or uterine/rectal prolapse.

To diagnose FHMS, the presence of two major, one major and two minor or four minor criteria is required

Excluded: Marfan syndrome due to the absence of damage to the lenses and ascending aorta; osteogenesis imperfecta in the absence of multiple bone fractures and “blue” sclera

Restricted mobility of the hip joints

The hip joints are the most powerful joints that are quite resistant to any impact. They act as an integral system. Stiffness of the hip joints appears due to a number of external and internal factors:

• mechanical injuries - fractures, bruises, dislocations;
• bursitis, accompanied by excessive production of synovial fluid, pain even at rest;
• inflammation of the tendons - tenditis, typical for athletes;
• arthritis of various origins - rheumatic, gouty, rheumatoid, tuberculous;
• intra-articular purulent infections;
• coxarthrosis.

All of these conditions require rapid diagnosis and adequate treatment. Elimination of unpleasant symptoms is carried out with medication or surgery.

Stiffness of the hip joints is observed in infants and appears mainly against the background of infectious diseases - mumps, rubella, meningitis, chickenpox, measles, ARVI. An inflammatory process develops in the joint, the most severe complication of which is pyogenic coxitis. Ignoring symptoms and improper treatment can lead to the death of a child.

Restriction in the hip joints of children may appear as a negative consequence of vaccination. In this case, antiallergic drugs come to the rescue. In any case, if there is an increase in temperature and pain in the leg, you should immediately consult a doctor.

How does joint stiffness occur?

The elasticity of articular elements is provided by collagen. This is a protein compound produced by fibroblasts - special connective tissue cells. Insufficient collagen synthesis leads to loss of physiological sliding of the articular head in the joint capsule.

The most common causes of joint stiffness are:

• active growth of bones, ligaments, tendons in childhood and adolescence;
• dehydration of the cartilage covering the articular heads of bones;
• muscle spasms that appear against the background of a sedentary lifestyle;
• diseases affecting the joints;
• inflammation of bone and cartilage tissue;
• physical exercise.

We must not forget that any stressful situations or psycho-emotional stress can cause pain and affect joint mobility. If the pathological condition is based on nervous symptoms, painkillers do not affect the patient. The problem should be solved by adjusting the regimen and taking sedatives.

Diagnostics

If your doctor thinks you may have joint hypermobility, the Beighton test is often used as a quick test to assess the range of motion in certain joints.

However, it cannot be used to confirm the diagnosis, as it is important to examine all joints.

Your doctor may also order blood tests and x-rays to rule out other conditions associated with joint pain, such as rheumatoid arthritis.

If you have other symptoms in addition to hypermobile joints, your GP may further evaluate your condition using the Brighton criteria, which can help determine whether you have joint hypermobility syndrome (JHS).

Shoulder stiffness

In the shoulder joint, stiffness is manifested by severe pain and a sharp limitation of motor activity. Contracture of the shoulder joints is classified depending on the underlying cause of the pathology:

• adhesive capsulitis;
• postoperative stiffness;
• post-traumatic contracture.

Post-traumatic stiffness is caused by fractures, dislocations, ruptures of the joint capsule and tendons. Fractures always entail erosion of bone tissue and subsequent limitations.

Adhesive capsulitis forms against the background of inflammatory processes. The disease often becomes chronic and leads to the formation of fibrous scar changes.

During operations on the upper limb girdle with the installation of metal structures, the limb is preserved, but the appearance of contracture is inevitable. Stiffness in the shoulder joint appears in women after a mastectomy.

Operating technique and the scale of intervention affect the functioning of the ligamentous apparatus, the level of skin sensitivity, and the innervation of the upper limb.

Stiffness in the joints of the fingers

Restrictions in finger mobility appear as a result of progressive arthritis, a chronic inflammatory process. The causes of stiff finger joints are:

• age-related processes associated with hormonal changes, slowing metabolism, menopausal processes;
• injuries;
• professional conditions: sewing, working with small parts, machines;
• hereditary predisposition;
• immune disorder;
• diabetes mellitus, gout;
• systemic pathologies: rheumatism, systemic lupus erythematosus;
• psoriasis.

The first symptoms of the disease do not cause concern in a person, since they are not accompanied by painful sensations. Morning stiffness is replaced by constant moderate pain. Over time, the joints become deformed and contractures form.

Ankle stiffness

About 50% of all patients with joint contractures turn to traumatologists for ankle joint stiffness. Changes in the functions of the ankle joints lead not only to limitation of motor activity. Against the background of contracture, destruction of the spine, changes in the shape of a healthy limb, flat feet, and atrophy of the lower leg muscles develop.

The most common causes of ankle stiffness are:

• physical exercise;
• sprains;
• tendon injuries;
• wearing a plaster cast;
• damage to nerve fibers;
• deforming arthritis;
• degenerative diseases.

It is important to remember that independent physical impact on the damaged ankle joint is unacceptable. Only a doctor should provide assistance to a patient.

Knee stiffness

Pathological changes in the knee joints cause discomfort during walking, incomplete extension or flexion of the knee. Any movement causes pain.

Stiffness in the knees appears as a consequence or symptom of various diseases:

• purulent-inflammatory lesions of joints;
• degenerative and dystrophic processes;
• burns;
• congenital weakness of muscles and ligaments;
• suffered meniscus injuries, dislocations, fractures;
• Bekhterev's disease, psoriasis;
• diabetes mellitus

People who are overweight and athletes who experience intense physical activity are prone to the formation of contractures.

Stiffness in the knee joints develops gradually and is accompanied by unpleasant symptoms that are constantly getting worse.

Stiffness of the jaw joints after dislocation

The temporomandibular joint is susceptible to dislocation due to rough mechanical impacts on the lower jaw. In some cases, unpleasant consequences lead to:

• yawn;
• scream;
• vomit;
• laughter;
• tooth extraction;
• taking dental impressions;
• gastroscopy;
• bronchoscopy;
• intubation;
• bad habits of chewing hard food with teeth, tearing packaging or opening bottles
• falling on the chin;
• direct blows to the lower jaw or temporal joint.

Stiffness of the jaw joints is observed with osteomyelitis, arthritis, and malocclusion. The condition is accompanied by increased salivation, impaired swallowing, changes in facial symmetry, and tension in the masticatory muscles.