Juvenile rheumatoid arthritis

Juvenile rheumatoid arthritis (JRA) - arthritis of an unknown cause, lasting more than 6 weeks, developing in children aged no older than 16 years with the exclusion of another joint pathology.

Depending on the type of classification, the disease has the following names: juvenile arthritis (ICD-10), juvenile idiopathic arthritis (ILAR), juvenile chronic arthritis (EULAR), juvenile rheumatoid arthritis (ACR).

ICD-10 code

• M08. Juvenile arthritis.
• M08.0. Juvenile (juvenile) rheumatoid arthritis (sero-positive or seronegative).
• M08.1. Juvenile ankidosis spondylitis.
• M08.2. Juvenile (juvenile) arthritis with systemic onset.
• M08.3. Juvenile (juvenile) polyarthritis (seronegative).
• M08.4. Pauziarticular Juvenile Arthritis.
• M08.8. Other juvenile arthritis.
• M08.9. Juvenile arthritis, unspecified.

Epidemiology of juvenile chronic arthritis

Juvenile rheumatoid arthritis is one of the most frequent and most disabling rheumatic diseases that occurs in children. The incidence of juvenile rheumatoid arthritis ranges from 2 to 16 people per 100,000 children under the age of 16 years. The prevalence of juvenile rheumatoid arthritis varies from 0.05 to 0.6% in different countries. Rheumatoid arthritis is often affected by girls. Mortality is 0.5-1%.

Adolescents have a very unfavorable situation with rheumatoid arthritis, its prevalence is 116.4 per 100 000 (in children under 14, it is 45.8 per 100 000), the primary incidence is 28.3 per 100 000 (in children under 14, 12.6 per 100 000).

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

Causes of juvenile chronic arthritis

For the first time juvenile rheumatoid arthritis was described at the end of the last century by two well-known pediatricians: the Englishman Stille and the Frenchman Schaffar. Over the next decades in the literature, this disease was referred to as the Still-Shaffar disease.

Symptomocomplex of the disease included: symmetrical damage of the joints, the formation of deformations, contractures and ankylosis in them; the development of anemia, enlarged lymph nodes, liver and spleen, sometimes the presence of febrile fever and pericarditis. Subsequently, in the 30-40s of the last century, numerous observations and descriptions of the syndrome of the syndrome revealed much in common between rheumatoid arthritis in adults and children, both in clinical manifestations and in the nature of the course of the disease. However, rheumatoid arthritis in children was also different from the disease with the same name in adults. In this connection, in 1946 two American researchers Koss and Boots proposed the term "juvenile (juvenile) rheumatoid arthritis". The nosological isolation of juvenile rheumatoid arthritis and adult rheumatoid arthritis was subsequently confirmed by immunogenetic studies.

What provokes juvenile rheumatoid arthritis?

[6], [7], [8], [9]

Pathogenesis of juvenile chronic arthritis

The pathogenesis of juvenile rheumatoid arthritis has been intensively studied in recent years. The development of the disease is based on the activation of both cellular and humoral immunity.

Pathogenesis of juvenile chronic arthritis

Symptoms of juvenile chronic arthritis

The main clinical manifestation of the disease is arthritis. Pathological changes in the joint are characterized by pain, swelling, deformities and restriction of movements, an increase in skin temperature over the joints. Children are most often affected by large and medium joints, in particular, knee, ankle, wrist, elbow, hip, less often - small hand joints. Typical for juvenile rheumatoid arthritis is the defeat of the cervical spine and maxillofacial joints, which leads to underdevelopment of the lower and, in some cases, upper jaw and the formation of the so-called "bird's jaw".

Symptoms of juvenile chronic arthritis

Classification of juvenile chronic arthritis

Three classifications of the disease are used: the classification of juvenile rheumatoid arthritis of the American College of Rheumatology (ACR), the classification of juvenile chronic arthritis of the European League against rheumatism (EULAR), the classification of juvenile idiopathic arthritis of the International League of Rheumatological Associations (ILAR).

Classification of juvenile chronic arthritis

[10], [11], [12], [13], [14]

Diagnosis of juvenile chronic arthritis

In the systemic version of juvenile rheumatoid arthritis, leukocytosis (up to 30-50 thousand leukocytes) with a neutrophilic shift to the left (up to 25-30% of stabic leukocytes, sometimes to myelocytes) is often detected, an increase in ESR to 50-80 mm / h, hypochromic anemia, thrombocytosis , an increase in the concentration of C-reactive protein, IgM and IgG in serum.

Diagnosis of juvenile chronic arthritis

[15]

Objectives of treatment of juvenile chronic arthritis

• Suppression of inflammatory and immunological activity of the process.
• Kupirovanie systemic manifestations and articular syndrome.
• Preservation of the functional capacity of joints.
• Prevention or slowing down the destruction of joints, disability of patients.
• Achieving remission.
• Improving the quality of life of patients.
• Minimizing the side effects of therapy.

Treatment of juvenile chronic arthritis

Prevention of juvenile chronic arthritis

Due to the fact that the etiology of juvenile rheumatoid arthritis is unknown, no primary prevention is performed.

Forecast

With a systemic version of juvenile rheumatoid arthritis in 40-50% of children is a favorable prognosis, there may come a remission lasting from several months to several years. However, the exacerbation of the disease can develop years after a persistent remission. In one-third of patients, the recurring course of the disease is continuously observed. The most unfavorable prognosis in children with persistent fever, thrombocytosis, prolonged therapy with corticosteroids. In 50% of patients, severe destructive arthritis develops, 20% have amyloidosis in adulthood, and 65% have severe functional insufficiency.

All children with an early debut of polyarticular seronegative juvenile arthritis have an unfavorable prognosis. In adolescents with seropositive polyarthritis, there is a high risk of developing severe destructive arthritis, disability according to the condition of the musculoskeletal system.

In 40% of patients with oligoarthritis with an early onset, destructive symmetrical polyarthritis is formed. In patients with late onset, the disease can be transformed into ankylosing spondylitis. 15% of patients with uveitis may develop blindness.

An increase in the level of C-reactive protein, IgA, IgM, IgG is a reliable sign of an unfavorable prognosis of the development of joint destruction and secondary amyloidosis.

Mortality in juvenile arthritis is low. Most of the deaths are associated with the development of amyloidosis or infectious complications in patients with a systemic version of juvenile rheumatoid arthritis, often resulting from prolonged glucocorticoid therapy. In secondary amyloidosis, the prognosis is determined by the possibility and success of the treatment of the underlying disease.

[16]