Juvenile rheumatoid arthritis - Causes, Symptoms, Diagnosis and Treatment
Juvenile rheumatoid arthritis - Progressive, destructive inflammatory joint disease in children, is under the age of 16 years, and combined with extra-articular pathology. The articular form of the disease is manifested by edema, deformity, contracture of large and small joints of the extremities, the cervical spine; systemic form is accompanied by general symptoms: high fever, polymorphous rash, generalized lymphadenopathy, hepatosplenomegaly, affecting the heart, lungs, kidneys. The diagnosis of juvenile rheumatoid arthritis is based on data from clinical, laboratory tests, X-rays and punctures of joints. When juvenile rheumatoid arthritis appointed NSAIDs, corticosteroids, immunosuppressive drugs, physical therapy, massage, physiotherapy.
Juvenile rheumatoid arthritis
Rheumatology. According to statistics, it is diagnosed in 005-0.6% of young patients worldwide. Usually pathology manifests itself not earlier than two years of age, with 1.5-2 times more common in girls. Juvenile rheumatoid arthritis rheumatica refers to a debilitating disease, often leads to disability at a young age.
Epstein-Barr virus, retroviruses), joint trauma, sun exposure or hypothermia, injection of protein drugs. Potential arthritogenic incentives may make collagen proteins (types II, IX, X, XI, oligomeric matrix protein of cartilage, proteoglycans). The important role played by the family and a genetic predisposition to the development of rheumatic disease (sublokusov carriage of certain HLA-antigens).
One of the leading pathogenesis of juvenile rheumatoid arthritis is a congenital or acquired disorders of immunity, leading to the development of autoimmune processes. In response to the impact of causal factors modify IgG (autoantigens), which induces the production of autoantibodies - rheumatoid factors. As part of the circulating immune complexes, the RF chain is launching a series of pathological reactions resulting in damage to the synovial membrane and vascular endothelial development in their purulent chronic inflammation exudative alterative, and then proliferative character.
It noted the formation of microvilli, lymphoid infiltration, proliferation of granulation tissue (pannus) and erosions on the surface of the articular cartilage, epiphyseal cartilage destruction and bone, joint space narrowing, atrophy of the muscle fibers. The progressive fibro-sclerotic joint disease occurs in children with seropositive and system variant of juvenile rheumatoid arthritis; It leads to irreversible changes in the joints, the development of subluxation and dislocation, contractures, fibrous and bony ankylosis, limited function of joints. Degradation of connective tissue and vascular changes also occur in other organs and systems. Juvenile rheumatoid arthritis can occur in the absence of the rheumatoid factor.
rheumatoid arthritis in adults, but differs from it in articular and extra-articular manifestations. The classification of the American College of Rheumatology (the AKP) identifies 3 types of juvenile rheumatoid arthritis: systemic, polyarticular, and oligo (pautsi-) articular (types I and II). On oligoarthritis type I accounts for up to 35-40% of cases, suffer mostly girls, the debut - under the age of 4 years. Oligoarthritis type II is seen in 10-15% of patients, mostly boys, the beginning - at the age of 8 years.
By immunological characteristics (the presence of rheumatoid factor), juvenile rheumatoid arthritis are divided into seropositive (RF +) and seronegative (RF-). According to the clinical and anatomical features distinguish:
The course juvenile rheumatoid arthritis can be slow, moderate or fast progressing. There are four degrees of activity of juvenile rheumatoid arthritis (high - III, the average - II, low - I, remission - 0) and grade 4 disease (I-IV) depending on the degree of joint function.
TMJ ( "bird jaw"). The defeat of the hip usually develops in the later stages of the disease.
There may be a low-grade fever, weakness, mild splenomegaly and lymphadenopathy, weight loss, growth retardation, lengthening or shortening of the limbs. The articular form of juvenile rheumatoid arthritis is often combined with rheumatoid lesions of the eye (uveitis, iridocyclitis), a sharp drop in visual acuity. Rheumatoid nodules are typical for RF-positive poliartricheskoy form of the disease occurring in older children having more severe, the risk of developing rheumatoid vasculitis, and Sjogren's syndrome. RF-negative juvenile rheumatoid arthritis occurs in every childhood is relatively easy for a rare form of rheumatoid nodules.
The system is characterized by a severe form of extra-articular manifestations: persistent febrile fever hectic nature, polymorphous rash on the limbs and trunk, generalized lymphadenopathy, Banti's syndrome, myocarditis, pericarditis, pleurisy, glomerulonephritis. Joint damage can occur during the initial period of systemic juvenile rheumatoid arthritis, or a few months later, while taking a chronic relapsing course. Still's syndrome is more common in children of preschool age, for it is characteristic of arthritis with the defeat of small joints. Wissler-Fanconi syndrome usually occurs in school age and proceeds with the dominance of large polyarthritis, in t. Ch. Hips without marked deformations.
Complications of juvenile rheumatoid arthritis are secondary amyloidosis of the kidneys, liver, myocardium, colon, macrophage activation syndrome can be fatal, cardiopulmonary failure, growth retardation. Oligoarthritis type I is accompanied by chronic iridocyclitis with the risk of vision loss, oligoarthritis type II - spondyloarthropathy. The progression of juvenile rheumatoid arthritis leads to permanent joint deformity with partial or full restriction of their mobility and early disability.
children's rheumatology and pediatric ophthalmology, laboratory studies (of Hb, ESR, the presence of RF, antinuclear antibodies), X-ray and MRI of the joints, joint puncture (artrotsenteza).
The criteria of juvenile rheumatoid arthritis are: opening up to 16 years; disease duration of more than 6 weeks; the presence of at least 2-3 attributes (symmetrical arthritis, deformation of small joints of the hands, the destruction of the joints, rheumatoid nodules, positivity for RF positive data synovial biopsy, uveitis).
X-ray stage juvenile rheumatoid arthritis is determined by the following criteria: I - epiphyseal osteoporosis; II - epiphyseal osteoporosis with narrowing of the joint space, single edge defects (Uzury); III - the destruction of cartilage and bone, numerous Uzury, subluxations of the joints; IV - the destruction of cartilage and bone with fibrous or bony ankylosis.
The differential diagnosis is carried out with juvenile ankylosing spondylitis, juvenile psoriatic arthritis, reactive and infectious arthritis, Ankylosing spondylitis, Reiter's disease, lupus, inflammatory bowel disease (Crohn's disease, Whipple's disease), bone tumors, acute leukemia.
Medical treatment of juvenile rheumatoid arthritis include symptomatic (anti-inflammatory) drugs are fast acting and pathogenetic (basic) tools. In the acute phase of the articular syndrome appointed NSAIDs (diclofenac, naproxen, nimesulide), if necessary - corticosteroids (prednisolone, betamethasone) into local and intra-articularly or in the form of pulse therapy. Prolonged use of basic drugs, immunosuppressants (methotrexate, sulfasalazine) reduces the need for symptomatic drugs, prevent progression, prolong remission improve prognosis and juvenile rheumatoid arthritis.
An important component of the treatment of juvenile rheumatoid arthritis is physical therapy, massage, physiotherapy (medicinal phonophoresis, mud, paraffin, ozokerite applications, UFO, laser therapy) and treatment by natural factors. When expressed deformation of joints and the development of severe ankylosis shown prosthetic joints.
Prediction and prevention of juvenile rheumatoid arthritis
Juvenile rheumatoid arthritis is a lifelong diagnosis. With adequate treatment and regular monitoring rheumatologist possible long-term remission without marked deformations and loss of joint function with a satisfactory quality of life (studies, work in the profession). exacerbation risk may persist for many years. More than a poor prognosis with early opening, continuously relapsing course, RF + polyarteritis, and systemic forms of juvenile rheumatoid arthritis, leading to the development of complications, severe restrictions of movement in the joints and disability. For the prevention of relapse of juvenile rheumatoid arthritis should avoid sun exposure, hypothermia, change of climate zone, limit contact with infectious patients, to exclude preventive vaccination and receiving immunostimulants.