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Bechterew's disease

 
, medical expert
Last reviewed: 11.04.2020
 
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Bechterew's disease is a chronic inflammatory disease of the spine (spondylitis) and sacroiliac joints (sacroiliitis), often occurring with affection of peripheral joints (arthritis) and enthesis (entesitis), and in some cases the eye (uveitis) and aortic bulb (aortitis).

Ankylosing spondylitis is more commonly known as Bekhterev's disease, although its fuller name is Striumpell-Bechterew-Marie's disease. This disease with autoimmune pathogenesis, has a chronic progressive course and can last for years and decades.

Ankylosing spondylitis, or Bechterew's disease, affects, as a rule, cartilaginous types of joints, mainly sternoclavicular, rib-breast, sacroiliac, hip and shoulder joints, as well as lesions of small intervertebral joints. Healthy joint tissues are gradually replaced with fibrous scar tissue, and joint stiffness develops.

trusted-source[1], [2], [3], [4], [5], [6]

Epidemiology

The prevalence of Bechterew's disease correlates with the frequency of detection of HLA-B27 in the population and varies (among adults) from 0.15% (Finland) to 1.4% (Norway). The disease develops mainly at the age of 20-30 years, and in men 2-3 times more often.

Quite often, Bekhterev's disease occurs at a young age, about 20-30 years, more often men fall ill. There are many theories of the development of this disease, but the genetic theory is most often preferred. It is believed that Bekhterev's disease has a hereditary predisposition due to the presence of the histocompatibility antigen HLA-B27, because of which the immune system perceives cartilaginous tissues as foreign, and directs its immune response to them, they cause inflammation, pain, restriction of motion and stiffness in the joints.

trusted-source[7], [8], [9], [10], [11], [12], [13], [14], [15], [16]

How does Bekhterev's disease develop?

The only common risk factor for the development of Bechterew's disease is a genetic predisposition. More than 90% of patients have HLA-B27 - one of the genes of class I of the main histocompatibility complex. A relationship was established between the frequency of this gene in different populations and the prevalence of the disease. However, the presence of HLA-B27 can not be considered a sign of mandatory occurrence of Bechterew's disease, and in most carriers of this gene the disease does not develop. According to family and twin studies, the proportion of HLA-B27 in predisposition to the development of Bekhterev's disease is no more than 20-50%. In addition, there are at least 25 alleles of HLA-B27 (B27001-B2723), differing from each other with only one nucleotide in the DNA composition and, correspondingly, 17 amino acid changes in the area of the recognition groove of the protein. Not all HLA-B27 alleles are associated with predisposition to Bechterew's disease (for example, B2706 in Southeast Asia or B2709 in Sardinia). Perhaps the development of the disease and in the absence of this antigen. Nevertheless, most hypotheses about the origin of Bechterew's disease are based on the assumption of the involvement of HLA-B27 in the pathogenesis of the disease.

There are two main hypotheses of the formation of the disease.

  1. The first of these is based on the assumption of the antigen presenting function of the protein encoded by HLA-B27. It is known that in this protein there are sites similar to the epitopes of some enterobacteria (in particular, Klebsiella, Enterobacter, Shigella, Yersinia), as well as Chlamydia trachomatis. It is believed that due to this similarity, autoimmune reactions may occur with the appearance of antibodies and cytotoxic T-lymphocytes to their own tissues (the phenomenon of molecular mimicry). Circulating antibodies cross-reacting with HLA-B27 and antigens of these microorganisms, as well as cytotoxic T-lymphocytes, were found in patients with AS. However, there is no clinical evidence of the role of these immune reactions in the development of Bechterew's disease.
  2. In accordance with the second hypothesis, it is assumed that for some unknown reason, there may be anomalies in the assembly of the heavy chain of the protein molecule HLA-B27 in the endoplasmic reticulum of cells. As a result, the conformationally altered molecules of this protein accumulate and degrade, which leads to the development of intracellular "stress" with excessive synthesis of proinflammatory mediators.

Nevertheless, on the basis of these hypotheses, it is not possible to explain the absence of Bechterew's disease in the majority of persons who have HLA-B27, as well as the predominant lesion of the spine, joints and entecies.

The peculiarity of the spinal cord injury in Bechterew's disease consists in the inflammation of various structures: bone tissue (osteitis), joints (intervertebral, arcuate, rib-vertebral), and enthesis (where fibrous discs attach to vertebral bodies, interstitial ligaments). In places of inflammation of the spine, chondroid metaplasia occurs, followed by ossification of these zones and ankylosing of the disturbed structures.

Arthritis in Bekhterev's disease is morphologically non-specific, but has a characteristic localization; almost always mark the defeat of the sacroiliac joints, often the pathological process is involved in the sternostem or rib-vertebra joints, as well as symphysis (symphysis of the sternum, pubic symphysis).

Bekhterev's disease is characterized by inflammation of enthesias (places of attachment of many ligaments, tendons, aponeuroses, capsules of joints to the bones, mainly in the region of the shoulder, hip, knee joints and heels), which have fibrous cartilage in their composition.

Symptoms of Bechterew's Disease

Very often there is a central form of the disease, when the spine is mostly affected. Bekhterev's disease develops imperceptibly for the patient himself, and for a long time can only make itself felt in the form of pain during exercise or with active movements. Pain first localises in the sacrum and gradually rises up the vertebral column. The patient's posture begins to change slowly, she starts taking the "supplicant's posture" after a while - the head leans forward, the chin approaches the sternum, the thoracic spine becomes more prominent posteriorly, the stoop appears, the arms are bent at the elbow joints, and the legs in the knee joints. For this form, as in other and for all other forms, there are night pains and pains at rest, and by the middle of the day the pain decreases, the person "paces" and the pains decrease significantly.

However, gradually in the spine there is a decrease in the volume of movements, it is difficult for a person to lean forward, to make a deflection backward, inclines to the sides. Over time, the gait begins to change significantly - this is due not only to the damage to the spine, but also to the involvement of the hip joints in the process.

With the development of ankylosis - the fusion of the joints in the spine - the head turns sideways to the side, to see what is happening behind, a person needs to turn around with the entire body. Bechterew's disease has one of the characteristic features - the disappearance of the lumbar curve and the appearance of a strong stoop in the thoracic region.

In addition, Bekhterev's disease also has extra-articular symptoms - defeat of the eyes, heart, kidneys. In general, the disease occurs in the form of exacerbation and remission phases, but it is still a chronic progressive disease.

Where does it hurt?

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Classification

The following forms of the disease are distinguished.

  • Idiopathic Bechterew's disease.
  • Secondary disease of Bechterew as one of the manifestations of other diseases from the group of seronegative spondyloarthritis (psoriatic arthritis, reactive arthritis, spondyloarthritis associated with ulcerative colitis with Crohn's disease).

The causes of Bechterew's disease are unknown.

trusted-source[17], [18], [19], [20], [21], [22], [23]

Screening

Screening to identify people who are predisposed to the development of Bekhterev's disease is currently considered unjustified. However, screening measures are recommended for the early diagnosis of Bechterew's disease among people aged less than 30 years with chronic low back pain who are at risk for developing the disease (for example, acute unilateral anterior uveitis or the presence of Bechterew's disease or other seronegative spondylitis in the relatives of the first degree of kinship).

In what cases can Bekhterev's disease be suspected?

Back pain is more than 3 months, especially if they increase after a long rest. Pain can radiate into the buttock, hamstring, groin, stiffness in the spine in the morning, muscle tension in the lumbar region, pain in the muscles of the chest, a significant increase in ESR - up to 30 mm / hour.

As a rule, with such complaints all patients get to a rheumatologist or vertebrologist. The establishment of the correct diagnosis depends on them. On how qualitatively the examination was carried out, complaints were collected and the correct methods of investigation were assigned, the early diagnosis of Bekhterev's disease and the purpose of further treatment depended. The earlier treatment was started, the more likely it is to prolong the patient's working period, to postpone the appearance of prolonged painful pains and the development of complications.

What diagnostic methods are needed?

This is an x-ray study of the spine, MRI - magnetic resonance imaging, clinical studies of blood and urine, biochemical blood test, blood for the detection of HLA-B27 antigen.

Bechterew's disease refers to chronic inflammatory diseases, it is characterized by the defeat of the sacroiliac, synovial (intervertebral and rib-transverse) and non-synovial (discovertebral) joints of the spine, as well as the attachment of ligaments and tendons to the bodies of the vertebrae and the bones of gas. The primary sacral lesions are sacroiliac joints, thoracolumbar and lumbosacral spine. Subsequently, changes can determine in all parts of the spine. The involvement of peripheral joints in the pathological process is moderately expressed. In this case, they speak of the peripheral form of Bekhterev's disease. Changes in the hip and shoulder joints are most common in comparison with other peripheral joints.

Sacral-ileal joints

For the diagnosis, Bekhterev's disease requires the presence of changes in the sacroiliac joints. The absence of sakroileitis causes great difficulties in verifying this disease and doubt the diagnosis. Very rarely changes in the spine can occur with the absence of convincing radiologic symptoms of sacroiliac joint damage. In this case, dynamic monitoring of changes in these joints and differential diagnosis with other diseases from the group of seronegative siondilloarthrites are required. Sacroiliitis with ankylosing spondylitis occurs at the earliest stages of the development of the disease and is characterized in typical cases by bilateral and symmetrical spreading.

Spine with ankylosing spondylitis

The primary lesions of the spine, especially in men, are the thoracolumbar and lumbosacral spine, in women in the early stages of the disease, the cervical spine can be affected. Anterior spondylitis associated with local erosive changes in the anterior region of the vertebral bodies and inflammation in the anterior longitudinal ligament of the spine leads to a decrease in the concavity of the vertebral body.

The result of these changes is the "quadraticization" of vertebral bodies typical for Bekhterev's disease, clearly visible on the lateral radiograph of the spine. These changes are found in the lumbar spine, as the thoracic vertebrae normally have a configuration approaching the right of the coal. Syndesmophytes vertically oriented bone ossicata, located outside the fibrous ring of the intervertebral disc. They prevail in the region of the anterior and lateral parts of the vertebral bodies and form bone bridges between the vertebral bodies. In the late stages of the disease, multiple syndesmophytes join together on a large extent and form the characteristic length of Bekhterev's disease "bamboo" spine. It is important to note the fact that syndesmophytes. Characteristic of ankylosing spondylitis, as well as enterogenic spondylitis, differ from syndesmophytes and osteophytes found at corners of vertebral bodies in other diseases. They have a clear, even contour, smoothly passing from one vertebral body to another. Osteophytes with deforming spondylosis - triangular in shape, have the shape of a "jug handle" up to 10 mm in length, located on the front and side corners of vertebral bodies. With diffuse idiopathic bone hyperostosis in the spine (Forestier syndrome) calcification of the anterior longitudinal ligament is observed over a large extent with the formation of coarse, deformed osteophytes at the anterior and lateral angles of vertebral bodies with a wall thickness of up to 4-6 mm and a length of up to 20-25 mm. And differ from syndesmophytes in AS, the width of which does not exceed 1-2 mm. In addition, with ankylosing hyperostosis there are no changes in the sacroiliac joints. Erosion of articular surfaces and narrowing of one or more intervertebral discs (spondylodiscites) are characteristic radiographic symptoms for Bechterew's disease. These changes can be divided into local and common. The outcome of spondylodiscitis can be calcification of the intervertebral disc, and if spondylodisitis is combined with destructive changes in the articulating vertebral bodies, then bone ankylosis of adjacent vertebrae can form. Lesions of intervertebral joints occur later, but the outcome of arthritis can also be ankylosing. In addition to the lesion of the anterior longitudinal ligament, ossification of the posterior longitudinal and interocular ligaments of the spine is detected. Erosions in the dentate process and atlanto-axial subluxation can be detected with Bechterew's disease, although with a lower frequency than with RA. Ankylosis can also be found in the area of the atlanto-axial joint. In other parts of the cervical spine, changes, if found, are identical to those found in the thoracolumbar spine.

A distinctive characteristic of radiological changes can be a combination of erosive and proliferative changes and the attachment of ligaments, which makes it possible to conduct differential diagnosis with other inflammatory and non-inflammatory diseases, diagnosing Bechterev's disease.

However, it should be noted that there are no specific diagnostic signs for the presence of ankylosing spondylitis, there is only a complex of symptoms and data of laboratory and other types of studies that allow excluding other diseases, such as rheumatoid arthritis, and reliably diagnose Bekhterev's disease.

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Treatment of Bechterew's Disease

Treatment for Bechterew's disease is only complex. This includes both medical therapy, and exercise therapy, and sanatorium treatment, and even, if necessary - surgical intervention. Medications are mainly aimed at arresting the pain syndrome and the removal of inflammatory phenomena. To this end, use non-steroidal anti-inflammatory drugs, such as, ibuprofen, indomethacin, diclofenac sodium, etc. But a large role is still assigned to exercise therapy and, in general, the motor activity of the sick person.

Physician LFK can offer the patient an individual set of exercises that will help to remove stiffness, increase the amount of movement, strengthen muscles, give more flexibility to the joints and relieve pain. In the beginning, the exercise will have to be done, overcoming the pain, but gradually it will become smaller, the volume of movements will increase, and the stiffness will decrease. Of course, exercise therapy is not a panacea, but its huge role in this disease can not be denied.

People who are diagnosed with Bechterew's disease, but who continue to maintain their physical form and those who lead an active lifestyle, can remain working for a much longer time and delay the development of complications.

More information of the treatment

Drugs

Prevention

It is not possible to prevent Bekhterev's disease. You can conduct medical genetic counseling to determine the risk of the disease in a child who is born from parents who are sick with the disease.

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