Bechterew's disease: symptoms

Symptoms of Bechterew's disease do not depend on gender or the presence of HLA-B27.

The formation of inevitable ankylosis of the spine with the formation and, in some cases, kyphosis of the cervical and/or thoracic regions (“petitioner’s pose”) is usually preceded by various symptoms of Bechterew’s disease over many years (usually tens of years).

Symptoms of Bechterew's disease begin before the age of 40, mainly in the third decade of life. Development of the disease after 40 is not typical, although other seronegative spondyloarthritides (usually psoriatic arthritis) may occur at this age. In about 25% of cases, the disease appears in childhood. Symptoms of Bechterew's disease do not always begin with signs of spondylitis or sacroiliitis. Several variants of the onset of the disease can be distinguished.

• Gradual development of inflammatory pain in the lower back and sacroiliac joints. In the first months and years, the pain may be unstable, spontaneously decrease or disappear for a while.
• The onset of peripheral arthritis (mainly hip, knee, ankle, foot joints) and enthesitis of various localizations (usually in the heel area). This onset of the disease is almost always observed in children and adolescents, less often in young adults. In some cases, arthritis occurs acutely after urogenital or intestinal infection and meets the criteria for reactive arthritis.
• The development of all changes in the musculoskeletal system may be preceded by acute recurrent anterior uveitis.
• In rare cases, mainly in children and adolescents, a persistent increase in temperature may come to the forefront of the clinical picture.
• The disease is known to debut with damage to the aortic bulb, aortic valve and/or the cardiac conduction system.

Ankylosis of the spine is sometimes almost painless, and ankylosis is discovered by chance on an X-ray taken for other reasons.

The symptoms of Bechterew's disease, which begins in childhood, are distinctive. Peripheral arthritis and/or enthesitis are almost always noted, sacroiliitis with severe pain and typical systemic symptoms of Bechterew's disease (anterior uveitis and other symptoms) are possible, but signs of damage to the spinal column are usually either absent or weakly expressed and develop only in adulthood, and their progression occurs slowly and typical speech genotype changes are formed later than usual.

The distinction between these variants of the onset of the disease is conditional. Most often, a combination (in various combinations) of signs of spondylitis, peripheral arthritis, enthesitis, uveitis and other symptoms of Bechterew's disease is observed.

Bechterew's disease has general symptoms. Among them, the most characteristic are general weakness and weight loss. Relatively rarely, mainly in children and adolescents, an increase in body temperature (usually subfebrile) is a concern.

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Uveitis

Symptoms of Bechterew's disease are characterized by acute (no more than 3 months), anterior, recurrent uveitis. Unilateral localization of inflammation is typical, but alternating damage to both eyes is also possible. Uveitis may be the first symptom of Bechterew's disease (sometimes preceding the appearance of other signs of the disease by many years) or occur simultaneously with damage to the joints and spine. With untimely or inadequate treatment, complications may develop in the form of pupillary synechia, secondary glaucoma and cataracts. In rare cases, inflammation of the vitreous body and edema of the optic nerve, ischemic neuropathy of the optic nerve (usually in combination with severe vitritis) are also detected. In some patients, posterior uveitis (usually joining the anterior) or panuveitis is possible.

Aortic and cardiac lesions

Symptoms of Bechterew's disease are often accompanied by signs of aortitis, aortic valve valvulitis and damage to the cardiac conduction system. According to echocardiography and ECG, the frequency of these disorders slowly increases with the duration of the disease, reaching 50% or more with a disease duration of 15-20 years. During the section, damage to the aorta and aortic valves is detected in 24-100% of cases. Changes in the aorta and heart are usually not associated with other clinical symptoms of Bechterew's disease, general disease activity, or the severity of damage to the spine and joints. Cardiovascular disorders are often not clinically manifested and are diagnosed only during a targeted examination, but in some patients, rapid (within a few months) development of critical consequences is possible (severe aortic insufficiency with left ventricular failure or bradycardia with attacks of loss of consciousness).

Typically, the aortic bulb is affected over approximately the first 3 cm, with involvement of the aortic cusps, the sinuses of Valsalva, and in some cases, adjacent structures such as the membranous portion of the interventricular septum and the anterior cusp of the mitral valve. As a result of inflammation with subsequent development of fibrosis, the walls of the aortic bulb thicken (mainly due to the adventitia and intima), especially behind and immediately above the sinuses of Valsalva, and dilation of the aorta occurs, sometimes with relative insufficiency of the aortic valve.

EchoCG reveals thickening of the aortic bulb, increased rigidity of the aorta and its dilation, thickening of the aortic and mitral valve cusps, and regurgitation of blood through the valves. A unique echocardiographic phenomenon is identified - local thickening (in the form of a ridge) of the left ventricular wall in the groove between the aortic valve cusps and the basal part of the anterior mitral cusp, histologically represented by fibrous tissue.

With regular ECG recording, various conduction disturbances are noted in approximately 35% of patients. Complete atrioventricular block occurs in 19% of patients. According to the electrophysiological study of the heart, the pathology of the atrioventricular node itself, rather than the underlying sections, is of primary importance in the genesis of conduction system dysfunction. Inflammatory lesions of the vessels feeding the conduction system have been described. The formation of bundle branch blocks and atrioventricular conduction disturbances may also be due to the spread of the pathological process from the membranous part of the interventricular septum to its muscular part. During daily ECG monitoring, significant variability of the QT interval was detected in patients, which may indicate myocardial damage.

There is evidence of the occurrence of disturbances (usually minor) of diastolic function of the left ventricle in approximately 50% of patients in relatively early stages of the disease. Myocardial biopsy performed in these patients in individual cases has shown a slight diffuse increase in interstitial connective tissue and the absence of inflammatory changes or amyloidosis.

In some patients, a slight thickening of the pericardium is detected (usually using echocardiography), which, as a rule, has no clinical significance.

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Kidney damage

A characteristic but not frequent symptom of Bechterew's disease is considered to be IgA nephropathy - glomerulonephritis caused by immune complexes containing IgA. Light microscopy reveals focal or diffuse proliferation of mesangial cells, and immunohistochemical examination reveals IgA deposits in the renal glomeruli. IgA nephropathy is clinically manifested by microhematuria and proteinuria. Less common is macrohematuria (tea-colored urine), which can be combined with upper respiratory tract infections. The course of IgA nephropathy varies, but is more often benign, without signs of progression of kidney pathology and impairment of their functions over a long period of time. However, gradual development of hypertension, increasing proteinuria, deterioration of renal function, and the formation of renal failure are also possible.

Approximately 1% of patients develop clinically evident amyloidosis with predominant renal involvement, which determines the outcome of the disease. Amyloidosis is considered to be a late symptom of Bechterew's disease. In serial biopsies of various tissues (for example, adipose tissue of the anterior abdominal wall) performed in patients with a relatively short duration of the disease, amyloid deposits were detected in approximately 7% of cases, but clinical manifestations of amyloidosis were noted at the time of morphological examination only in a small proportion of these patients.

Drug-induced renal dysfunction is possible, most often caused by taking NSAIDs. According to some data, patients have an increased incidence of urolithiasis.

Although Bechterew's disease often does not show neurological symptoms, in some cases (usually with a long-term course of the disease) spinal cord compression develops due to subluxations in the median atlantoaxial joint, as well as as a result of traumatic fractures of the vertebrae. In some patients and in the late stages of the disease, cauda equina syndrome may occur. This is caused by a specific pathological process - the appearance of predominantly dorsal diverticula of the arachnoid membrane of the spinal cord, compressing the spinal roots. Since significant destruction of the legs and plates of the arches of the lower lumbar vertebrae is usually detected at the same time, it is assumed that the inflammatory process can spread to the membranes of the splint brain, contributing to the formation of diverticula. In this case, a complex of symptoms characteristic of narrowing of the spinal canal at the lower lumbar level is noted: weakness of the urinary bladder sphincters (the manifestation of which is similar to the symptoms of prostate adenoma), impotence, decreased skin sensitivity in the lower lumbar and sacral dermatomes, weakness of the corresponding muscles, and decreased Achilles reflex. Pain syndrome does not often bother patients. Myelography reveals a typical picture: an expanded cavity of the dura mater ("dural sac") filled with arachnoid diverticula.

Bechterew's disease: symptoms of spondylitis

The inflammatory process can be localized in the anatomical structures of various segments of the spine, but usually begins in the lumbar region. The so-called inflammatory pain is characteristic: constant aching pain, increasing at rest (sometimes at night) and accompanied by morning stiffness. With movement and taking NSAIDs, the pain and stiffness decrease. Sciatica pain is not characteristic. Along with pain, movement restrictions develop, and in several planes. The severity of the pain syndrome in the spine (especially at night) usually corresponds to the activity of the inflammation. Although the symptoms of Bechterew's disease at the beginning of the pathological process may be unstable, spontaneously decrease and even disappear, a gradual tendency for pain to spread up the spine is typical.

When examining the patient at first, there may not be any changes, except for pain during extreme movements in one or another section of the spine, flattening of the lumbar lordosis, limitations of movement in several directions and hypotrophy of the paravertebral muscles. Pain during palpation of the spinous processes of the vertebrae and paravertebral muscles is usually not noted. Fixed contractures of the spine (cervical kyphosis and thoracic hyperkyphosis), atrophy of the back muscles usually develop only in the late stages of the disease. Scoliosis of the spine is not typical.

Over time (usually slowly), the pain spreads to the thoracic and cervical spine, causing limited movement. Pain in the thoracic spine due to inflammation of the costovertebral joints can radiate to the chest, and also increase with coughing and sneezing.

The development of ankylosing of the spine usually leads to a decrease in pain. However, even with the presence of a "bamboo stick" X-ray picture, the inflammatory process may continue. In addition, in later stages of spondylitis, pain may be caused by complications such as compression fractures of the vertebrae and their arches, which occur with minor injuries (falls), and usually in those segments that are located between the ankylosed vertebrae. Arch fractures are difficult to diagnose on conventional X-rays, but they are clearly visible on tomograms.

An additional source of neck pain and movement restrictions in the cervical spine may be subluxations in the medial atlantoaxial joint. They develop as a result of the destruction of the joint between the anterior arch of the atlas and the odontoid axial vertebra and the ligamentous apparatus of this area and are characterized by the displacement of the axial vertebra backwards (rarely upwards), which can lead to compression of the spinal cord with the appearance of corresponding neurological symptoms,

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Bechterew's disease: symptoms of sacroiliitis

Sacroiliitis necessarily develops with Bechterew's disease (only isolated exceptions are described) and is asymptomatic in most patients. Approximately 20-43% of adult patients are bothered by peculiar sensations of this lesion alternating (moving from one side to the other for one or several days) pain in the buttocks, sometimes severe, leading to lameness. These symptoms of Bechterew's disease usually do not last long (weeks, rarely months) and pass on their own. During examination, local pain in the projection of the sacroiliac joints can be detected, but this physical sign, as well as various tests previously proposed for the clinical diagnosis of sacroiliitis (Kushelevsky tests and others), are unreliable.

Conventional radiography is of decisive importance in the diagnosis of sacroiliitis. It is preferable to take a general image of the pelvis, since in this case it is possible to simultaneously assess the condition of the hip joints, pubic symphysis and other anatomical structures, changes in which can contribute to the detection and differential diagnosis of the disease.

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Bechterew's disease: arthritis symptoms

Peripheral arthritis is observed throughout the disease in more than 50% of patients. In about 20% of patients (especially in children) it may begin with peripheral arthritis.

Any number of joints may be affected, but monoarthritis or asymmetrical oligoarthritis of the lower extremities, mainly the knee, hip and ankle joints, are most often detected. Less frequently, an inflammatory process is noted in the temporomandibular, sternoclavicular, sternocostal, costovertebral, metatarsophalangeal and shoulder joints, but these symptoms of Bechterew's disease are also considered characteristic of the disease. A feature of the disease (and other seronegative spondyloarthritides) is the involvement of cartilaginous joints (symphyses) in the pathological process. Damage to the pubic symphysis, which is clinically rare (although sometimes very pronounced), is usually diagnosed only by X-ray examination.

Symptoms of arthritis in Bechterew's disease, which do not have characteristic morphological features (including from the side of the cerebrospinal fluid), can begin acutely, resembling reactive arthritis. Chronic, persistent course of arthritis is more often noted, but spontaneous remissions are also known. In general, arthritis in patients is characterized by a lower rate of progression, development of destruction and functional disorders than, for example, in RA.

However, peripheral arthritis of any localization can become a serious problem for the patient due to severe pain, destruction of articular surfaces and dysfunction. Coxitis, often bilateral, is considered the most prognostically unfavorable. It often develops in children. At first, low-symptom and even asymptomatic damage to this joint is possible. According to ultrasound data, effusion in the hip joints in patients occurs more often than clinical manifestations of coxitis. There are radiological features of coxitis: rare presence of periarticular osteoporosis and marginal erosions, development of marginal osteophytes of the head, appearing both in combination with narrowing of the joint space and cysts of the head of the femur and / or acetabulum, and in isolation. Formation of bone ankylosis is possible, which rarely occurs with coxitis of other etiologies.

Peripheral arthritis is often associated with enthesitis. This is especially true for the shoulder joint, where symptoms of enthesitis at the attachment sites of the rotator cuff muscles to the humeral tuberosities (with the development of marginal erosions and bone proliferation) may prevail over the symptoms of arthritis of the shoulder joint.

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Bechterew's disease: symptoms of enthesitis

Symptoms of inflammation of entheses in the disease are natural. Localization of enthesitis may be different. Clinically, enthesitis usually manifests itself clearly in the area of the heels, elbows, knees, shoulders and hip joints. The underlying bone (ostitis) with the development of erosions and subsequent ossification and bone proliferation may be involved in the inflammatory process, or it may spread to tendons (for example, tendinitis of the Achilles tendon) and their sheaths (tenosynovitis of the flexors of the fingers with the appearance of a clinical picture of dactylitis), aponeuroses (plantar aponeurosis), joint capsule (capsulitis) or adjacent synovial sacs (bursitis, for example, in the area of the greater trochanter of the femur). A subclinical course of enthesopathies is also possible, which is typical for damage to the entheses of the interspinous ligaments of the spine, tendons of the muscles attached to the wings of the iliac bones.

A chronic pathological process in the area of entheses, accompanied by destruction of the underlying bone tissue, and then excessive bone formation, is the basis for the appearance of such peculiar radiographic changes characteristic of the disease (and other seronegative spondyloarthritides), such as extra-articular bone erosion, subchondral osteosclerosis, bone proliferation (“spurs”) and periostitis.

When the disease occurs, damage to other organs and systems is observed, among which the most common are uveitis, changes in the aorta and heart.

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