Syndrome of hyper mobility of joints

Description:

A peculiar disease which symptoms are the increased distensibility of skin, looseness of joints with tendency to their incomplete dislocations, fragility of vessels and easy vulnerability of skin that is regarded as manifestation of system inferiority of connecting fabric. After descriptions of Elersav of 1901 and Danlov 1908 is a disease was called Elersa-Danlo's syndrome ("hyper elastic skin", "the rubber person").

Symptoms of the Syndrome of hyper mobility of joints:

The clinic of SGMS is diverse and includes both the joint, and extraarticular manifestations in general reflected in the mentioned Braytonovsky criteria of a syndrome of GMS.

Essential help in diagnosis is given by attentive collecting the anamnesis. The characteristic fact in the history of life of the patient is his special sensitivity to exercise stresses and tendency to frequent injuries (stretchings, incomplete dislocations of joints in the past) that allows to think of insolvency of connecting fabric. Beytona revealed by method the excess volume of movements in joints supplements actually clinical forms of manifestation of SGMS.

Joint manifestations

Arthralgia and mialgiya. Feelings can be burdensome, but are not followed by the seen or palpated changes from joints or muscles. The most frequent localization — knee, ankle, small joints of brushes. At children the expressed pain syndrome in the field of a hip joint answering massage is described. Pain degree of manifestation is often influenced by an emotional condition, weather, a phase of a menstrual cycle.
The acute posttraumatic joint or circumarticular pathology which is followed by a synovitis, tenosinovity or a bursitis.
Periartikulyarny defeats (tendinites, epicondylitis, other entezopatiya, bursitis, tunnel syndromes) occur at patients with SGMS more often than in population. Arise in response to unusual (unusual) loading or the minimum injury.
Chronic mono - or the polyarticulary pain in some cases accompanied with the moderate synovitis provoked by an exercise stress. This manifestation of SGMS most often leads to diagnostic mistakes.
Repeated dislocations and incomplete dislocations of joints. Typical localizations — humeral, patello-femolyarny, metacarpophalangeal joints. Sprain in an ankle joint.
Development of an early (premature) osteoarthrosis. It can be both a true nodular polyosteoarthrosis, and the secondary damage of large joints (knee, coxofemoral) arising against the background of the accompanying orthopedic anomalies (flat-footedness, not recognizable dysplasia of hip joints).
Dorsodynias. Thoracalgias and lyumbalgiya are widespread in population, especially at women 30 years therefore it is difficult to draw an unambiguous conclusion on communication of these pains with hyper mobility of joints are more senior. However the spondylolisthesis is authentically connected with GMS.
The symptomatic longitudinal, cross or combined flat-footedness and its complications: medial tenosinovit in an ankle joint, valgus deformation and secondary arthrosis of an ankle joint (longitudinal flat-footedness), a zadnetaranny bursitis, a talalgia, "natoptysh", "molotkoobrazny" deformation of fingers, Hallux valgus (metatarsus latus).
Extraarticular manifestations. These signs are natural as the main structural protein the collagen which is initially participating in the described pathology is also present at other basic fabrics (a fascia, a derma, a wall of vessels).

Excess distensibility of skin, its fragility and vulnerability. Striya, not connected with pregnancy.
The varicosis beginning in young years.
Prolapse of the mitral valve (before introduction to broad practice in the 70-80th years of an echocardiography many patients with a syndrome of GMS were observed at the rheumatologist with the diagnosis "rheumatism, the minimum degree of activity" in connection with complaints to joint pains and the noise in heart connected with a prolapse of valves).
Hernias of various localization (umbilical, inguinal, white line of a stomach, postoperative).
Vesceral ptosis — a stomach, kidneys, a uterus, a rectum.
Thus, at survey of the patient with suspicion on SGMS, and it is each patient of young and middle age with a noninflammatory joint syndrome, it is necessary to pay attention to possible accessory signs of a system dysplasia of connecting fabric. Knowledge of phenotypical manifestations of a syndrome of Marfan and imperfect bone formation allows to exclude these hereditary diseases. In that case if strong skin and vascular indications are found (hyper elasticity of skin and spontaneous formation of bruises without coagulopathy signs), it is lawful to speak about Elersa-Danlos's syndrome. Open is a question of differential diagnosis of a syndrome of high-quality GMS and the "softest", hyper mobile type of a syndrome of Elersa-Danlos. By means of Braytonovsky criteria it cannot be made what authors specially mention; in both cases moderate involvement of skin and vessels takes place. For this purpose, for other syndrome the biochemical marker is not known. The question remains open and, apparently, will be resolved only with detection of a specific biochemical or genetic marker for the described states.

Considering wide circulation of constitutional GMS in population, especially among youth, would be wrong to explain all joint problems at this category of persons only with hyper mobility. Existence of GMS does not exclude at all a possibility of development in them of any other rheumatic disease to which they are subject with the same probability, as well as persons with a normal volume of movements in joints.

Thus, the diagnosis of a syndrome of GMS becomes reasonable when other rheumatic diseases are excluded, and the available symptoms correspond to the clinical signs of a syndrome which are logically supplemented by detection of excess mobility of joints and/or other markers of generalized involvement of connecting fabric.

Reasons of the Syndrome of hyper mobility of joints:

The acquired excess mobility of joints is observed at ballet dancers, athletes and musicians. Long repeated exercises lead to sprain and capsules of separate joints. In this case local hyper mobility of a joint (joints) takes place. Though it is obvious that in the course of professional selection (dances, sport) the persons which are initially differing in constitutional flexibility have clear advantage, the fitness factor undoubtedly takes place. Changes in flexibility of joints are observed also at a number of morbid and physiological conditions (an acromegalia, a hyper parathyroidism, pregnancy). Generalized GMS is a characteristic sign of a number of the hereditary diseases of connecting fabric including Marfan's syndrome, imperfect bone formation, Elersa-Danlos's syndrome. These are rare diseases. In practice the doctor should deal with patients from the isolated GMS not connected with trainings and in some cases the weakness of connective tissue structures which is combined with other signs much more often. Almost always it is possible to establish family character of the observed GMS and the accompanying pathology that testifies to the genetic nature of the observed phenomenon.

Treatment of the Syndrome of hyper mobility of joints:

Treatment of the patient with a syndrome of GMS depends on a specific situation. A variety of manifestations of a syndrome assumes also the differentiated approach to each certain patient. An important point is the explanation in an available form of the reasons of its problems with joints ("weak sheaves") and belief of the patient that it has no serious illness threatening with inevitable disability. At moderate arthralgias of it it is enough. Recommendations to exclude loadings, defiant pains and discomfort in joints will be useful. Non-drug methods, and first of all — optimization of a way of life are solving in treatment of the expressed pains. It assumes reduction in compliance of loadings and a threshold of their portability by this patient. It is necessary to minimize possibilities of injuries that includes vocational guidance and an exception of game sports.

At persistent one or several joint pains use elastic orthoses (kneecaps, etc.). Timely correction of the revealed flat-footedness is very important. At the same time from the doctor elementary podologichesky knowledge is required — the form and rigidity of insoles is defined individually, success of treatment in many respects depends on it. Quite often it is possible to cope with persistent arthralgias of knee joints only this way.

In ensuring stability of a joint an essential role is played not only by sheaves, but also surrounding a muscle joint. If by exercises it is impossible to affect a condition of the copular device, then strengthening and increase in force of muscles — a real task. The gymnastics at a syndrome of GMS has feature — it includes so-called "isometric" exercises at which there is a considerable muscle tension, but the volume of movements in joints is minimum. Depending on localization of a pain syndrome (knee joints), a shoulder girdle, a back etc. recommend to strengthen muscles of hips. Swimming is useful.

Medicamentous therapy is applicable as a symptomatic treatment at arthralgias. As pains at GMS syndrome generally have the noninflammatory nature, it is quite often possible to see total absence of effect of use of non-steroidal anti-inflammatory drugs.

In this case the bigger result can achieve reception of analgetics (paracetamol, трамадол). Intra joint introduction of corticosteroids in the absence of signs of a synovitis is absolutely inefficient.

At periartikulyarny defeats (tendinites, entezopatiya, a bursitis, tunnel syndromes) treatment tactics practically does not differ from that at ordinary patients. In moderately expressed cases it is ointments with non-steroidal anti-inflammatory drugs in the form of applications or compresses; in more persistent — local introduction of small doses of the glucocorticosteroids which are not possessing mestnodegenerativny action (suspension of crystals of Methylprednisolonum, betamethasone). It should be noted that efficiency of local therapy by corticosteroids to a large extent depends on correctness of statement of the topical diagnosis and technology of implementation of the procedure.