Juvenile ankylosing spondylitis

Juvenile spondyloarthritis, or Bekhterev's disease, is a group of clinically and pathogenetically similar rheumatic diseases of childhood, including juvenile ankylosing spondylitis, juvenile psoriatic arthritis, reactive (post-enterocolitis and urogenital) arthritis associated with HLA-B27 antigen, Reiter's syndrome, enteropathic arthritis in inflammatory diseases intestinal (regional enteritis, ulcerative colitis). In this group, undifferentiated spondyloarthritis is also distinguished (for the designation of those clinical situations in which the patient has only individual manifestations characteristic of spondyloarthritis, and there is no entire symptom complex of a particular nosological form, which, in fact, serves as the stage of disease formation and in natural evolution, as a rule , leads to the development of juvenile ankylosing spondylitis or psoriatic arthritis).

A so-called syndrome of seronegative arthrosis / enthesopathy (SEA syndrome), introduced into the practice of pediatric rheumatology in 1982 by Canadian scientists A. Rosenberg and R. Petty, for distinguishing juvenile rheumatoid arthritis, is characteristic of the juvenile onset of undifferentiated spondylitis. Acute anterior uveitis is also often considered as one of the variants of juvenile spondyloarthritis, with the exception of other causes of ophthalmic pathology. The group of juvenile spondyloarthritis includes rare syndromes associated with skin changes (pustullet, acne) and osteitis (SAPHO syndrome, chronic recurrent multifocal osteomyelitis), characteristic for children and adolescents to a greater extent than for adults.

General characteristics of juvenile spondylitis:

• the predominant incidence of males;
• features of the joint syndrome, different from rheumatoid arthritis according to clinical characteristics, localization and prognosis;
• absence of rheumatoid factor in blood serum;
• frequent involvement in the pathological process of the spine;
• high carrier frequency of HLA-B27 antigen;
• the tendency to family aggregation for HLA-B27-associated diseases.

Despite the pathogenetic validity of combining the listed diseases into the group of juvenile spondyloarthritis, the use of this term in everyday medical practice is fraught with certain problems. Thus, the International Classification of Diseases of the Tenth Revision (ICD-10) does not provide a separate rubric for the entire spondyloarthritis group, therefore the use of the generalized term "juvenile spondylitis" or the diagnosis of "undifferentiated spondylitis", coded in M46, in medical documents and forms of statistical reporting data on the prevalence of rheumatic diseases in children. Juvenile ankylosing spondylitis according to ICD-10 is considered in M08 "Juvenile arthritis" and corresponds to item M08.1. Juvenile psoriatic arthritis and enteropathic arthritis are classified under M09, and reactive arthropathies are classified under M02. In practice, a significant number of children and adolescents with spondyloarthritis are diagnosed with "juvenile chronic arthritis" (M08.3, M08.4) and even "juvenile rheumatoid arthritis" (M08.0), which is explained by the long period of nonspecific juvenile onset of spondyloarthritis clinical manifestations, the so-called prespondyl stage of the disease. Juvenile rheumatoid arthritis occupies a central place in the group of juvenile spondyloarthritis, being its prototype. This disease is usually considered to be identical to ankylosing spondyloarthritis), although significant features of the clinical picture of juvenile rheumatoid arthritis give reason to discuss its nosological isolation. The central position of AC / SAS in the spondyloarthritis group is due to the fact that any of the diseases included in this group can not only have certain features of ankylosing spondylitis, but also lead to its development, virtually indistinguishable from idiopathic ankylosing spondylitis.

Juvenile ankylosing spondylitis is a chronic inflammatory disease of the peripheral joints, tendon-ligament apparatus and spine that occurs before the age of 16, characterized by the predominant incidence of males, a tendency to family aggregation and association with HLA-B27 antigen.

The synonym for AU / SAC, gradually disused in the scientific literature, is the term "Bechterew's disease".

History of the study of juvenile spondylitis

Ankylosing spondylitis is a disease that has been known to mankind for thousands of years. Archaeological excavations and study of bone remains of ancient people and animals have found reliable signs of this suffering. The priority of the first scientific description of ankylosing spondylitis is owned by Irish physician Bernard O'Connor, who 300 years ago in 1691 and 1695. On the skeleton of a person accidentally found in the cemetery, gave a detailed description of morphological changes inherent in ankylosing spondylitis.

Anatomical studies for a long time preceded the clinical study of ankylosing spondylitis and only in the 19 century in the literature began to appear single clinical descriptions of the disease. Already then in the reports Benjamin Travers. Lyons and Clutton gave examples of the onset of ankylosing spondylitis in children and adolescents.

Article V.M. Bechterew "The stiffness of the spine with its curvature as a special form of the disease," published in 1892 in the journal Vrach, initiated the isolation of ankylosing spondylitis in a separate nosological form and introduction of the term "Bechterew's Disease" into use by doctors. Sometimes in medical literature there is also the term "Bekhterev-Strympel-Marie's disease", which includes the names of two more scientists who were at the beginning of the study of ankylosing spondylitis. So. Strumpell in 1897 showed that the basis of the disease is a chronic inflammatory process in the spine and sacroiliac joints, and not in "areas adjacent to the hard shell of the spinal cord," as VMMozhin assumed. Bechterew. Marie In 1898, he described the pores of the isoelic form of the disease, thereby combining the defeat of the axial skeleton and peripheral joints in a single process. Actually the term "Ankylosing spondylitis", reflecting the morphological basis of the disease in its natural course, was proposed by Frenkel in 1904.

The publication in 1942 of Scott SG's book "A monograph on adolescent spondylitis or ankylosing spondylitis" attracted doctors' attention to the study of ankylosing spondylitis with a juvenile onset. Until the mid-1980s, this problem was covered in the literature only by single publications and only the last decade was marked by a surge of interest in the study of juvenile ankylosing spondylitis, which was reflected in the works of many foreign authors (Ansell V., Burgos-Vargas R., Bywatftrs E. , Cassidy J., Harrier R., Jacobs B., Job-Deslandre C, Khan M., Petty R., Ramus-Rem us C, Rosenberg A., Shaller J. Et al.). In our country, a significant contribution to the study of the problem of juvenile ankylosing spondylitis was made in the 80-90s by the cycle of publications by Professor AA.

Epidemiology of juvenile spondylitis

Exact data on the prevalence of juvenile spondylitis are absent, they are based, mainly, on mathematical calculations. If we take into account that among adults of the white race, manifest ankylosing spondylitis occurs at a frequency of 2: 1000 and above, and among all cases of ankylosing spondylitis 15-30% falls on the juvenile onset, the prevalence of juvenile spondylitis should be 0.03-0, 06%. In clinical practice, juvenile spondyloarthritis is recognized much less often due to a significant delay in the development of the main pathognomonic manifestation of spondyloarthritis - spine injury. The results of long-term follow-up observations in assessing nosological outcomes in adult patients show that juvenile spondyloarthritis accounts for up to 25-35% of all cases of juvenile arthritis. Foreign epidemiological studies, which show that the disease of every 3-4 patients among patients with juvenile arthritis can be referred to the group of juvenile spondylitis, confirm this data. For example, according to the results of a major epidemiological study conducted in the United States, the incidence of juvenile spondyloarthritis (excluding USPA) is 1.44 per 100 000 population, while the incidence of juvenile arthritis as a whole is 4.08 per 100 000.

As well as ankylosing spondylitis of adults, juvenile spondyloarthritis and especially juvenile spondyloarthritis are diseases with pronounced sexual determination. Boys get sick 6-11 times more often than girls, although this ratio is likely to be 5: 1 or 3: 1, since in women, both adults and children, ankylosing spondylitis often occurs sub-manifested, and in severe clinical forms more often , than in men, are diagnosed with rheumatoid arthritis.

[1], [2], [3], [4], [5], [6]

Causes of juvenile spondylitis

The etiology of juvenile spondylitis is unknown, the cause of the development of this pathology is obviously polyethiologic.

The modern level of knowledge is limited by the understanding of predisposing factors and individual links of pathogenesis. In the origin of this disease, the combination of genetic predisposition and environmental factors is important. Among the latter, the most important role is played by infections, primarily some strains of Klebsiella, other enterobacteria, and also their associations interacting with antigenic structures of the macroorganism, for example, HLA-B27 antigen. The high carrier frequency of this antigen (70-90%) in patients with juvenile spondyloarthritis, compared with 4-10% in the population, confirms the role of HLA-B27 in the pathogenesis of the disease.

Causes and pathogenesis of juvenile ankylosing spondylitis

[7], [8], [9], [10], [11], [12], [13]

Symptoms of juvenile spondylitis

Because juvenile ankylosing spondylitis is a prototype for the entire group of juvenile spondyloarthritis, its inherent clinical manifestations occur with all the diseases of this group in the form of separate symptoms or their combinations.

60-70% of children become ill with juvenile spondyloarthritis over the age of 10, however, in rare cases an early onset (up to 7 years) occurs, the debut of juvenile ankylosing spondylitis at the age of 2-3 years is described. The age of the onset of the disease determines the spectrum of clinical manifestations in the onset of juvenile ankylosing spondylitis and the pattern of its further course.

Symptoms of juvenile ankylosing spondylitis

Classification of juvenile spondylitis

Modern concepts of the relationship between diseases classified as spondyloarthritis may reflect the classification proposed in 1997 by Professor E.R. Agababovoy.

Classification of juvenile ankylosing spondylitis

[14], [15]

Diagnosis of juvenile spondylitis

When diagnosing juvenile spondylitis, one should be guided by existing classification and diagnostic criteria based on a combination of anamnestic data, clinical manifestations and the necessary minimum of additional studies:

• Radiography of the pelvis;
• X-ray, MRI and CT of the spine and peripheral joints (in the presence of clinical data);
• examination of the ophthalmologist on a slit lamp in order to verify the presence and nature of uveitis;
• functional examination of the heart;
• immunogenetic analysis (HLA-B27 typing).

Diagnosis of juvenile ankylosing spondylitis

[16], [17], [18], [19]

Treatment of juvenile spondylitis

A special emphasis in the treatment of juvenile spondylitis should be given to teaching the patient a rational regimen, developing a correct functional stereotype, carefully designed complex of therapeutic gymnastics (LFK), aimed at limiting static loads, maintaining proper posture, and maintaining a sufficient volume of movements in the joints and spine. It is important to orient the patient to perform daily physical exercises in order to prevent progressive kyphosis.

How is juvenile ankylosing spondylitis treated?

Prevention of juvenile spondylitis

Primary prevention is not carried out, however, given the high risk of accumulation of related diseases in families, genetic counseling and NLA-B27 typing for unaffected siblings may be considered appropriate.