Joint hypermobility syndrome - Causes, Symptoms and Treatment .MF .
hypermobility syndrome (GS) - a systemic disease of the connective tissue, which is characterized by joint hypermobility (HMS), combined with complaints from the locomotor system and / or internal and external phenotypic signs of connective tissue dysplasia in the absence of any other rheumaticdisease.
Symptoms of joint hypermobility syndrome are diverse and can mimic other more common diseases of the joints.Due to the lack of familiarity with this pathology physicians, and in some cases even rheumatologists and orthopedists often the correct diagnosis is not established.Traditionally, medical attention is drawn to the detection limit range of motion in the affected joint, and not the definition of excessive range of motion.The more that the patient will never report the excessive flexibility, since he lives together with her childhood and, moreover, are often convinced that it is rather a plus than a minus.Typical two diagnostic extremes: in one case, in the absence of objective evidence of pathology
Constitutional joint hypermobility is defined in 7-20% of the adult population.Although the majority of patients first complaints occur in the teenage period of life, symptoms can appear at any age.Therefore, the definition of "symptomatic" or "asymptomatic" HMS rather relative and reflect only the status of the individual with hypermobility syndrome in a certain period of life.
reasons hypermobility of joints
Acquired excessive mobility of joints seen in ballet dancers, athletes and musicians.Long-term repeated exercises lead to stretching of the ligaments and capsules of certain joints.In this case, there is a local joint hypermobility.While it is clear that in the course of professional qualification (dances, sports) persons initially differing constitutional flexibility, have a distinct advantage, fitness factor is undoubtedly the case.Changes to the flexibility of joints are also seen in a number of pathological and physiological states: acromegaly, hyperparathyroidism, pregnant.
Generalized joint hypermobility is a characteristic feature of a number of inherited connective tissue disorders, including Marfan syndrome, osteogenesis imperfecta, Ehlers-Danlos syndrome.It is a rare disease.In practice, the doctor more often have to deal with patients with isolated hypermobility of the joints, is not related to training and, in some cases combined with other signs of weakness of connective tissue structures.
almost always possible to establish the family character of the observed symptoms and comorbidities, indicating that the genetic nature of this phenomenon.It is noticed that hypermobility syndrome is inherited through the female line.
Diagnostics joint hypermobility syndrome
Among the many proposed ways to measure the range of motion in the joints was general recognition Beighton method, which is a nine-point scale, measures the ability of the subject to perform five movements (four pairs of legs and one for the torso and hips).Beighton proposed a simplified modification of the previously known method of Carter and Wilkinson.
movements criteria Baton
1. Passive extension of the little finger brush over 90 °.
2. Passive pressing the thumb to the inside of the forearm.
3. hyperextension of the elbow more than 10 °.
4. hyperextension of the knee more than 10 °.
5. The front slope of the body with palms touching the floor with straight legs.
It's simple and takes little time screening procedure and is used by doctors.
joint mobility standards for healthy people were determined on the basis of a number of epidemiological work.The degree of mobility of joints distributed in the population as a sine curve.
usual for Europeans is on the Beighton score from 0 to 4. But the average "normal" range of motion of joints is significantly different in age, sex and ethnic groups.In particular, during the examination of healthy persons in Moscow at the age of 16-20 years more than half of women and more than a quarter of men demonstrated a degree GMR greater than 4 points on the Beighton.Thus, in the absence of complaints on the part of the musculoskeletal system, the excess joint mobility in comparison with the average can be regarded as a constitutional feature, and even the age norm.In this regard, pediatric no generally accepted standards of joint mobility - the rate varies significantly during the growth of the child.
Braytonovskie criteria so called benign syndrome HMS (1998) are shown below.These criteria given value and extra-articular manifestations of weakness of connective tissue structures, which is indicative of HMS syndrome and in those with a normal range of motion in the joints (usually refers to older persons).
Criteria joint hypermobility syndrome
to establish common hypermobility is a numerical score of 1 point means the pathologic overextension in one joint on one side.The maximum value of the index, taking into account the two-way location - 9 points (8 - 4 for the first item and 1 - for the 5th paragraph).Score from 4 to 9 points is regarded as a state of hypermobility.
• Score on a scale Beighton 4 or more (at the time of inspection or in the past)
• Arthralgia for more than 3 months in four or more joints
• Score on a scale Beighton 1-3 (for people older than 50 years)
• Arthralgia 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 more than two locations(epicondylitis, tenosynovitis, bursitis)
• Marfanoidnost (tall, thin, the ratio of arm span / height & gt; 1,03, the ratio of upper / lower body segment & lt; 0,83, arachnodactyly)
• Abnormal skin: subtlety giperrastyazhimost,striae, atrophic scars
• Ocular signs: overhanging eyelids or myopia or antimongoloidnaya fold
• Varicose veins or hernia or uterine prolapse / rectum.
SGMS for diagnosis requires the presence of two large, one large and two small, or four minor criteria. excluded: Marfan syndrome by the absence of lesions of the lens and the ascending aorta;osteogenesis imperfecta by the absence of multiple bones and "blue" sclera fractures
Symptoms joint hypermobility syndrome
The basis of the pathogenesis of HS is a hereditary defect in collagen, accompanied giperrastyazhimostyu and decrease the mechanical strength of connective tissue structures (including ligaments, enthesis, tendons)leading to micro-traumas subluxation and articular apparatus (including the spine).
Syndrome Symptoms varied and includes both articular and extra-articular manifestations, generally recognized in the criteria mentioned GMR Braytonovskih syndrome.
essential help in diagnostics renders careful history taking.A characteristic fact in the history of the patient's life is his special sensitivity to physical stress and susceptibility to frequent injuries (sprains, subluxations of the joints in the past) that allows you to think about the inconsistency of the connective tissue.Tapped by Beighton excessive range of motion in the joints actually complements the clinical manifestations of the syndrome of hypermobility of joints.
Articular simptomy and potential joint hypermobility syndrome oslozhneneiya
arthralgia, and myalgia. Debut arthralgia accounts at a young age, especially females.Feelings can be painful, but they were not visible or palpable changes of the joints or muscles.The most frequent localization - knee, ankle, small joints of hands.The children described severe pain in the hip, is responsible for a massage.The degree of severity of pain often affects the emotional state of the weather, the phase of the menstrual cycle.
Acute traumatic articular or periarticular pathology , accompanied by synovitis, tenosynovitis and bursitis.
periarticular lesions (tendinitis, epicondylitis, other enthesopathies, bursitis, carpal tunnel syndrome) occur in patients with SGMS more often than in the population.There are in response to the extraordinary (unusual) load or minimal trauma.
Chronic mono- or polyarticular pain , in some cases accompanied by a mild synovitis, provoked by physical exertion.This is a manifestation SGMS most often leads to diagnostic errors.The cause of pain is to change the sensitivity of the proprioceptors to the load bearing joints in the background articular hypermobility.
Repeated dislocations and subluxations joints.Typical localization - humerus, patella-femolar, metacarpophalangeal joints.A sprain in the ankle.
Development of early (premature) osteoarthritis .It can be like a true nodular polyosteoarthrosis and secondary damage of large joints (knee, hip) that occurs on a background of concomitant orthopedic anomalies (flat, unrecognized hip dysplasia).
Pain in the back.Torakalgii and lumbalgia distributed in the population, especially in women over 30 years, so it is difficult to make an unambiguous conclusion about the relationship of pain with joint hypermobility.However it spondylolisthesis significantly associated with HMS.
Symptomatic longitudinal, lateral or combined flat and its complications: the medial tenosynovitis in the ankle area, valgus deformity and secondary osteoarthritis of the ankle joint (longitudinal flat feet), zadnetaranny bursitis, talalgiya, "corns", "hammer" finger deformity, Halluxvalgus (lateral flat).
Lack connective fixing the spine apparatus under the influence of adverse factors (long-term non-physiological position, the difference in the length of the lower limbs, wearing a bag on one shoulder) entails compensatory development spinal deformities (scoliosis), followed by overexertion musculo-ligamentous spinal structures and the emergence ofpain.
extra-articular manifestations of the syndrome.
These attributes are natural, as the main structural protein collagen, is primarily involved in the described pathology is also present in other supporting tissues (fascia, dermis, vascular wall).
- Excess skin extensibility, its fragility and vulnerability.
- striae, are not related to pregnancy.
- Varicose veins, starting at a young age.
- Mitral valve prolapse (before introduction into wide practice in 70-80s echocardiography many patients with the syndrome have been observed in HMS rheumatologist diagnosed with "rheumatism, the minimum level of activity" in connection with complaints of pain in the joints and heart murmurs relatedwith valve prolapse).
- hernias of various localization (umbilical, inguinal, white line of the abdomen, postoperative).
- omission of internal organs - stomach, kidney, uterus, rectum.
Thus, when viewed from a patient suspected of having hypermobility syndrome, and that each patient young and middle age with non-inflammatory articular 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 eliminates these hereditary diseases.In that case found clear skin, and cardiovascular symptoms (skin giperelastichnost and spontaneous bruising with no signs of coagulopathy), right to speak of Ehlers-Danlos syndrome.The open question is the differential diagnosis of benign joint hypermobility syndrome, and the most "soft", hypermobility type of Ehlers-Danlos syndrome.With Braytonovskih criteria make it impossible, as the authors specifically mention;in both cases there is a mild skin and vascular involvement.Neither for one nor for the other syndrome is not known biochemical marker.The question remains open and is, apparently, only allowed detection of specific genetic or biochemical marker for the described conditions.
Given the widespread constitutional hypermobility of the joints in the population, especially among young people, it would be wrong to explain all the joint problems in this category of persons only hypermobility.The presence of hypermobility syndrome does not exclude the possibility of them any other rheumatic disease, they are subject to just as likely as those with a normal range of motion in the joints.
Thus, the diagnosis of joint hypermobility syndrome becomes justified when excluded other rheumatic diseases and symptoms you are having consistent clinical signs of the syndrome, logically complemented identifying excessive mobility of joints and / or other markers of generalized involvement of connective tissue.
Complications hypermobility syndrome
1. Recurrent subluxations in the ankle joint.
2. Break the meniscus.
3. Frequent bone fractures.
4. Acute or recurrent shoulder subluxation, patella,
metacarpophalangeal, temporomandibular joints.
5. Traumatic arthritis.
3. The syndrome of the shoulder rotator cuff.
5. Episodic juvenile arthritis (synovitis) of the knee (with no evidence of systemic inflammatory response).
6. nonspecific arthralgia.
8. Back pain.
9. Chondromalacia patella.
12. Dysfunction of the temporomandibular joint.
13. Carpal and tarsal tunnel syndrome.
15. thoracic outlet syndrome.
17. Raynaud's Syndrome.
18. Delayed motor development (in children).
19. Congenital hip dislocation.
Treatment hypermobility syndrome sutavov
treatment of a patient with hypermobility syndrome depends on the situation.A variety of manifestations of the syndrome and suggests a differentiated approach to each individual patient.It is important to understand the root cause of the problems - the "weak link", it is not a serious disease and disability at no adequate way of life is not threatened.At moderate arthralgia enough to eliminate the load, causing pain and discomfort in the joints.
decisive in the treatment of pain are expressed in non-drug methods, and first of all - the optimization of lifestyle.This involves harmonizing load and the threshold of tolerance of a given patient.It is necessary to minimize the possibility of injuries that includes vocational guidance and exclusion sports game.
When persistent pain in one or more joints, flexible splints (knee pads, etc. N.) Providing an artificial limitation of motion.Very important is the timely correction of the detected flat feet.The shape and stiffness of the insoles is determined individually by the success of this treatment depends on many things.Often unable to cope with persistent arthralgias knee only in this way.
significant role in ensuring the stability of the joint is played not only ligaments surrounding the joint and muscles.If by exercise influence on the state of the ligamentous apparatus is not possible, strengthening and increasing muscle strength - the real challenge.