Bechterew's disease: symptoms

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

The formation of an imminent ankylosis of the spine with the formation and a number of cases of cervical and / or thoracic kyphosis (the "petition posture") is usually preceded by a variety of symptoms of Bekhterev's disease for many years (more often dozens of beds) .

Symptoms of Bechterew's disease begin at the age of 40, mainly in the third decade of life. The development of the disease after 40 years is atypical, although at this age other seronegative spondyloarthritis (usually psoriatic arthritis) may occur. Approximately in 25% of cases the disease appears in childhood. Not always symptoms of Bechterew's disease begin with signs of spondylitis or sacroiliitis. There are several options for the onset of the disease.

• Gradual development of inflammatory pains in the lower back and sacroiliac joints. In the first months and years of pain can be unstable, spontaneously diminished or for a while to pass.
• The appearance of peripheral arthritis (mainly hip, knee, ankle joints, joints of the feet) and enthesitis of various localization (more often in the heel area). Such a onset of the disease is almost always observed in children and adolescents, less often in young adults. In some cases, arthritis arises sharply after a urogenital or intestinal infection and meets the criteria for reactive arthritis.
• The development of all changes on the part of the musculoskeletal system may be preceded by an acute recurrent anterior uveitis.
• In rare cases, mainly in children and adolescents, a persistent increase in temperature may come to the fore in the clinical picture.
• Diseases of the disease are known from damage to the bulb of the aorta, the aortic valve and / or the conduction system of the heart.

Ankylosing of the spine is sometimes hardly accompanied by pain, and ankylosis is detected accidentally on an X-ray taken for other reasons.

The peculiarity of the symptoms of Bechterew's disease, beginning in childhood, differ. Virtually always mark peripheral arthritis and / or zheszity, sakroileitis with severe pain and typical systemic symptoms of Bechterew's disease (anterior uveitis and other symptoms) are possible, but signs of lesion of the spine are usually either absent or mild and develop only in adulthood, and their progression is slow and typical speech, genomic changes are formed later than usual.

Allocation of these variants of the onset of the disease is conditional. Most often observed combination (in various combinations) of the signs of spondylitis, peripheral arthritis, zntesitis, uveitis and other symptoms of Bekhterev's disease.

Bechterew's disease has common symptoms. Among them, the most common are weakness, weight loss. Relatively rare, mainly in children and adolescents, is concerned about fever (usually subfebrile).

[1], [2], [3], [4]

Uveitis

Symptoms of Bechterew's disease are characterized by acute (no more than 3 months), anterior, recurrent uveitis. Typical one-sided localization of inflammation, but it is possible and alternate damage to both eyes. Uveitis may be the first symptom of Bechterew's disease (sometimes outstripping the appearance of other signs of the disease for many years) or proceed concurrently with joint and spine involvement. In case of untimely or inferior treatment, complications such as synechia of the pupil, secondary glaucoma and cataract may develop. In rare cases, also the inflammation of the vitreous humor and edema of the optic nerve, ischemic neuropathy of the optic nerve (usually in combination with a severe vitriate). Individual patients may have a posterior uveitis (usually joining the anterior) or a panoveitis.

Aortic and cardiac damage

Symptoms of Bechterew's disease are often accompanied by signs of aortitis, valvulitis of the aortic valve and damage to the conduction system of the heart. According to EchoCG and ECG, the frequency of these disorders slowly increases as the duration of the disease increases, reaching 50% or more with a duration of the disease of 15-20 years. In the section, the lesions of the aorta and valves of the aorta are detected in 24-100% of cases. Changes in the aorta and heart are usually not associated with other clinical symptoms of Bechterew's disease, the overall activity of the disease, the severity of the lesion of the spine and joints. Cardiovascular disorders are not clinically manifested more often, and they are diagnosed only with a purposeful examination, however, in some patients, the development of critical consequences (expressed aortic insufficiency with left ventricular failure or bradycardia with loss of consciousness) can be rapid (within several months).

Characteristic is the destruction of the aortic bulb for approximately the first 3 cm with the involvement of the aortic valves, the sinuses of Valsalva, and in a number of cases, such adjacent structures as the membranous part of the interventricular septum and the anterior valve of the mitral valve. As a result of inflammation followed by the development of fibrosis, the walls of the aortic bulb thicken (mainly due to adventitia and intima), especially behind and immediately above the sinuses of Valsalva, and aortic dilatation occurs, sometimes with relative aortic valve insufficiency.

With echocardiography, thickening of the aortic bulb, increased stiffness of the aorta and its dilatation, thickening of the aortic and mitral valve flaps, regurgitation of blood through the valves are detected. Isolate a kind of echocardiographic phenomenon - a local thickening (in the form of a crest) of the left ventricle wall in the groove between the valves of the aortic valve and the basal part of the anterior mitral valve, histologically represented by fibrous tissue.

With regular ECG registration, various conduction disorders are noted in approximately 35% of patients. Complete atrioventricular blockade occurs in 19% of patients. According to the electrophysiological study of the heart, the pathogenesis of the atrioventricular node itself, rather than the underlying parts, is of primary importance in the genesis of disturbances in the function of the conducting system. An inflammatory lesion of the vessels feeding the conducting system is described. The formation of block bundles of the bundle of the bundle and violations of atrioventricular conduction can also be caused by the spread of the pathological process from the membranous part of the interventricular septum to its muscular part. With daily monitoring of ECG in patients, a significant variability of the QT interval was observed. Which may indicate the defeat of the myocardium.

There is information about the occurrence of violations (usually small) of the diastolic function of the left ventricle in about 50% of patients in the relatively early stages of the disease. Myocardial biopsy performed in these patients in some cases showed a small diffuse increase in interstitial connective tissue and the absence of inflammatory changes or amyloidosis.

In some patients, a small thickening of the pericardium is detected (usually with the help of echocardiogram), which, as a rule, does not have clinical significance.

[5], [6], [7], [8], [9], [10], [11], [12]

Renal damage

A characteristic but not frequent symptom of Bekhterev's disease is IgA-nephropathy - glomerulonephritis, caused by immune complexes containing IgA. With light microscopy, focal or diffuse proliferation of mesangial cells is detected, and in immunohistochemical studies, IgA deposits in the renal glomeruli are detected. IgA-nephropathy is clinically manifested by microhematuria and proteinuria. Less marked macrogematuria (urine tea color), which can be combined with infections of the upper respiratory tract. The course of IgA-nephropathy is different, but more often it is benign, with no signs of progression of kidney pathology and violation of their functions for a long time. However, gradual development of hypertension, the increase of proteinuria, impairment of renal function, formation of renal failure are possible.

Approximately 1% of patients develop clinically pronounced amyloidosis with primary renal disease, which determines the outcome of the disease. It is believed that amyloidosis is a late symptom of Bechterew's disease. Serial biopsies of various tissues (for example, adipose tissue of the anterior abdominal wall) were performed in patients with a relatively short duration of the disease, amyloid deposits were found in about 7% of cases, but clinical manifestations of amyloidosis were noted at the time of morphological examination in only a small part of these patients .

Possible drug disorders of the kidneys, more often due to the intake of NSAIDs. According to some data, patients have increased frequency of urolithiasis pathologies.

Although Bekhterev's disease often does not manifest neurological symptoms, in some cases (usually with a prolonged course of the disease), spinal cord compression develops as a result of subluxation in the mid-Atlantic joint and as a result of traumatic vertebral fractures. In some patients and late stages of the disease, cauda equina syndrome may occur. This leads to a peculiar pathological process - the appearance of predominantly dorsal diverticulae of the spinal cord of the spinal cord, compressing the spinal roots. Since both the destruction of the legs and plates of the lower lumbar vertebrae are usually detected at the same time, it is suggested that the inflammatory process can spread to the bushell casings, 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: the weakness of the sphincters of the bladder (manifestation of which is similar to the symptoms of prostate adenoma), impotence, a decrease in skin sensitivity in the lower lumbar and sacral dermatomes, weakness of the corresponding muscles, and reduction of the achilles reflex. Pain pain syndrome awaits patients not often. Myelography reveals a typical picture: an enlarged cavity of the dura mater ("dural sack") filled with arachnoidal diverticula.

Bechterew's disease: spondylitis symptoms

The inflammatory process can be localized in the anatomical structures of different segments of the spine, but usually begins in the lumbar region. Pain of the so-called inflammatory character is typical: worried constant aching pain, increasing at rest (sometimes at night) and accompanied by morning stiffness. When walking and receiving NSAIDs, pain and stiffness decrease. Ischialgic pain is not characteristic. Along with pain, restrictions on movements develop, and in several planes. The severity of the pain syndrome in the spine (especially at night) usually corresponds to the activity of inflammation. Although the symptoms of Bechterew's disease at the onset of the pathological process may be unstable, spontaneously diminished and even disappear, a typical gradual tendency to spread the pain up the spine is typical.

When examining a patient at first, there may not be any changes, except for pain in extreme movements in this or that part of the spine, a smoothness of the lumbar lordosis, restrictions of movements in several directions and hypotrophy of the invertebral muscles. Morbidity in palpation of spinous processes of the vertebrae and paravertebral muscles, as a rule, do not occur. Fixed contractures of the spine (cervical kyphosis and hyperkypophysis of the thoracic region), 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) pain spreads to the thoracic and cervical spine, causing restriction of movement. Pain sensations in the thoracic spine due to inflammation of the rib-vertebral joints can be irradiated to (the ore cage, and also strengthened by coughing and sneezing.

The development of ankylosing of the spine usually leads to the subsidence of the pain syndrome. However, even with an X-ray picture of a "bamboo stick", the inflammatory process can continue. In addition, in later stages of spondylitis, complications such as compression fractures of the vertebrae and their arches that occur with minor injuries (falls), and usually in those segments that are between the ankylosed vertebrae, can cause pain. Fractures of the arches are difficult to diagnose on conventional radiographs, but they are clearly visible on the tomograms.

An additional source of pain in the neck, restrictions of movements in the cervical spine may be subluxations in the mid-Atlantic atlas joint. They develop due to the destruction of the joint between the anterior arch of the atlas and the tooth of the axial vertebra and the ligamentous apparatus of this region and are characterized by the displacement of the axial vertebrae posteriorly (rarely upward), which can lead to compression of the spinal cord with the appearance of appropriate neurologic symptoms,

[13], [14], [15]

Bechterew's disease: symptoms of sacroiliitis

Sacroiliitis necessarily develops with Bekhterev's disease (only single exceptions are described) and in most patients it is asymptomatic. Approximately 20% of 43% of adult patients are concerned with the 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 limp. These symptoms of Bechterew's disease last usually not for long (weeks, rarely months) and passes independently. On examination, local soreness can be detected in the projection of the sacroiliac joints, but this physical sign, as well as the various tests offered earlier for the clinical diagnosis of sakroileitis (Kushelevsky's tests and others), are unreliable.

Critical in the diagnosis of sacroiliitis is the usual radiography. It is preferable to take a survey of the pelvis, because in this case it is possible to simultaneously assess the condition of the hip joint, pubic symphysis and other anatomical structures, the changes of which can contribute to the detection and differential diagnosis of the disease.

[16], [17], [18]

Bechterew's disease: symptoms of arthritis

Peripheric arthritis throughout the disease is noted in more than 50% of patients. Approximately 20% of patients (especially children) can start with peripheral arthritis.

Possible damage to any number of any joints, but more often monoarthritis or asymmetric oligoarthritis of the lower extremities, mainly knee, hip and ankle joints. Less often, the inflammatory process in temporomandibular, sternoclavicular, sternocostal, rib-vertebral, metatarsophalangeal and humeral joints is noted, but these symptoms of Bechterew's disease are also considered characteristic of the disease. A feature of the disease (and other seronegative spondylitis) is the involvement of the cartilaginous joints (symphysis) in the pathological process. The defeat of the pubic symphysis, which is clinically rare (although sometimes very brightly), is usually diagnosed only with radiographic examination.

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

Nevertheless, peripheral arthritis of any localization can become a serious problem for the patient due to severe pain, destruction of the articular surfaces and impaired function. The most prognostically unfavorable are the coke, often bilateral. It often develops in those who are sick in childhood. At first, there may be an asymptomatic and even asymptomatic lesion of this joint. According to ultrasound, the effusion in the hip joints in patients occurs more often than the clinical manifestations of the cocksite. There are roentgenological features of the cocksite: a rare presence of periarticular osteoporosis and marginal erosions, development of marginal osteophytes of the head, appearing both in combination with narrowing of the joint gap and cysts of the head of the femur and / or acetabulum, and in isolation. It is possible to form bone ankylosis, which rarely occurs with coxites of another etiology.

Peripheral arthritis is often combined with enthesitis. This is especially true for the shoulder joint region, where the symptoms of enthesitis at the points of attachment of the muscles of the rotator cuff of the shoulder to the tubercles of the humerus (with the development of marginal erosion and bone proliferation) can prevail over the symptoms of arthritis of the shoulder joint.

[19], [20], [21]

Bechterew's disease: symptoms of enthesitis

Symptoms of inflammation of enthesis with the disease is natural. The localization of enthesites can be different. Clinically distinctly, enthesites are usually found in the heels, elbows, knees, shoulder and hip joints. In the inflammatory process, the underlying bone (ostitis) can be involved with the development of erosion and subsequent ossification and bone proliferation, or it can spread to tendons (for example, tendon of the calcaneal tendon) and their membranes (flexor flexor tenosynovitis with the appearance of a clinical picture of dactylitis), aponeurosis (plantar aponeurosis ). Capsule of joints (capsulitis) or nearby synovial bags (bursitis, for example, in the region of a large trochanter of the femur). The subclinical course of enthesopathy is also possible, which is characteristic for the defeat of the enthesis of interstitial ligaments of the spine, the tendons of the muscles, attached to the wings of the ileal bones.

The chronic pathological process in the field of entesis is accompanied by the destruction of the underlying bone tissue, and then by excessive bone formation, it is the basis for the appearance of such peculiar radiographic changes characteristic of the disease (and other seronegative spondyloarthritis), as extraarticular bone erosion, subchondral osteosclerosis, bone proliferation ("spurs" ) and periostitis.

When the disease is observed, the defeat of other organs and systems, among which the most frequently observed uveitis, changes in the aorta and heart.

[22], [23], [24]