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Among the rheumatic diseases of childhood Yura takes on the prevalence of first
place. The disease is observed in different regions of the globe with a frequency of
0,05 to 0,6% in the population. Primary morbidity also varies widely, ranging from 6
to 19 cases per 100 000 child population.
ETIOLOGY
Etiology Jurassic hitherto unknown. Among his reasons for considering a set of
different environmental factors (viral and bacterial infection, trauma, joint, chill,
insolation, the introduction of protein drugs, etc.). The basis of an inadequate response
in patients with Yura is their "hypersensitivity to various environmental factors (EM
packages), resulting in a complex immune response that leads to the development of
progressive disease. Plays a role of family and inherited predisposition to rheumatoid
diseases.Research over the past decades have reported an association with the
presence of Jurassic patients with DR-locus HLA DR4 with a predominance of
patients with systemic forms of the disease and DR5 - with predominantly articular
variant of the disease.
PATHOGENESIS
The pathological process begins in the Jurassic in the synovial membrane of joints
microcirculation disturbances and destruction of cells lining the synovial
membrane. In response to the above changes in the patient's body formed by changing
the IgG, which are perceived by their own immune system as autoantigens.
Immunocompetent cells, including plasma cells of the synovial joint membranes in
response to generate AT - anti-IgG. These AT, called rheumatoid factor, the presence
of complement interacts with the autoantigen, and there is a formation of immune
complexes. CEC have damaging effects on both vascular endothelium and in the
surrounding tissue. Primarily affected joint synovium, resulting in arthritis develops.
In the synovial fluid and joint tissues, while cytokines produced excessive amounts of
macrophage origin - IL1 and IL6, tumor necrosis factor (TNF) wasps. IL1 induces
inflammation and destroy cartilage. This same property FNOoe. IL6 promotes
overproduction of acute phase proteins of inflammation - C-reactive protein and
fibrinogen. There is the further activation of enzyme systems that destroy
cartilage. Increased vascular tumors, or angiogenesis, arising as a result of action on
tissue cytokine also enhances the degradation of cartilage.
In the process of inflammation in the tissues of the joint formed by a large number of
cells forming the so-called pannus, or a cloak that covers the surface of the articular
cartilage, thus impeding the normal processes of metabolism and increasing
destruction of bone and cartilage entities.
Pathomorphology
When a biopsy of the synovial membrane in the initial period of illness reveal villous
hypertrophy and hyperplasia of the surface layer. Inflammation in the joint with the Jurassic, as
in adult patients, leads to the erosion and destruction of cartilage. However, these processes in
children develop more slowly and in a smaller percentage of cases. Typically, the number and
depth of erosion correspond to the depth and severity of the process. Simultaneously with the
pathological process in the joints of children watching swollen lymph nodes due to nonspecific
follicular hyperplasia. In children with Jurassic, as in adults, in all bodies may develop vasculitis,
non-specific morphological features. There are also atrophy of muscle fibers. The most
pronounced changes in the muscles surrounding the affected joints.
Clinical picture
The clinical picture of Jurassic diverse. Disease onset may be acute or subacute. At
the beginning of the acute usually increases body temperature, there is pain and then
swelling in one or more joints, usually symmetrical. However, the symmetry of the
lesions sometimes becomes apparent immediately, but within a few days or weeks
after onset of illness. Are affected, usually large joints - knees, ankles, Lu-
chezapyastnye, but sometimes from the very onset of the disease affects the small
joints of hands and feet (metatarsophalangeal, interphalangeal) (Fig. 13-1 and 13.2
inset). Typical of Jurassic defeat the joints of the cervical spine. All the joints are
severely painful, swollen, and in rare cases, the skin around them giperemiro-Wan.
The body temperature rises gradually and can reach 38-39 "C. It is often in the skin of
the trunk and extremities appeared polymorphic allergic rash, increased peripheral
lymph nodes, liver and spleen. In general, a blood test reveal anemia, often
neutrophils-tion leukocytosis with a shift to the left of leukocyte formula, erythrocyte
sedimentation rate increased to 40-60 mm / h, increasing the concentration of Ig,
mainly IgG.
Subacute onset characterized by less bright symptoms. Arthritis usually begins with a
single joint - the knee or ankle (Figure 13.3 inset). The joint swells, breaks down its
function, sometimes even without severe pain. The child's gait changes, and children
under 2 years stay away. Observe the so-called morning stiffness in the joints, which
is manifested in the fact that the patient feels after a night's sleep for some time,
difficulty in movements in the joints and self-service. He had barely stood up, his gait
slowed.Morning stiffness may last from several minutes to 1 hour or more. The
process for a long time may be limited to one of the joints (rheumatoid
monoartrit). This form of disease, particularly for girls of preschool age, often
accompanied by rheumatoid lesion of eye - rheumatoid uveitis, unilateral or
bilateral. Rheumatoid uveyte address all the membrane of the eye, resulting in sharply
falling acuity up to complete his loss, sometimes within six months. In rare cases, the
development of rheumatoid uveitis may precede articulate the process that is
extremely difficult to timely diagnosis.
Subacute onset may occur with involvement in the process a few joints - usually 2-
4. This form of disease called oligoartiku-lar. Joint pain may be moderate, as well as
pleural changes. The process may involve, for example, two ankle and one knee, and
vice versa. Body temperature did not rise, poliadenit moderate. This form Jurassic
proceeds more benign, with less frequent exacerbations.
Later, when the disease progresses, there may be two main forms - mainly articular
and articular-visceral in the ratio of 65-70% and 35-30% respectively.
Articular-visceral form
Articular form
When the articular form of progression Jurassic leads to resistant strains of joints with
partial or complete restriction of mobility in them. Up to 25% of children were
disabled (Figure 13.4 and 13.5 inset).
CLASSIFICATION
The main forms of Jurassic represented in the working classification of disease (the
domestic version, Tab. 13-4). In addition, the currently widespread Anglo-American
classification of Jurassic (Table 13-5).
DIAGNOSIS
Jurassic Diagnosis is often difficult, especially in the early stages of the disease. In
Russia, the following diagnostic criteria. Clinical signs
4. Contractures of joints.
5. Peritendinitis or bursitis.
6. Muscle atrophy.
7. Morning stiffness.
9. Rheumatoid nodules.
12. Narrowing of joint cracks, bone erosion, ankylosis of the joints (Figure 13.6 inset).
I. System Form
Subtype, observed mainly in girls with onset at an early age, the presence of antinuclear factor, the absence of rheumatoid factor, absence of HLA-B27
and the high risk of further eye damage
Subtype, observed mainly in boys, with onset in middle and older age, primary lesion of the lower extremities, the presence of HLA-B27 and the absence
of rheumatoid factor and antinuclear
Subtype observed among all age groups, the lack of rheumatoid factor, antinuclear factor and HLA-B27
Also used radiographic criteria for changes in the joints on the classification of the
American radiologist Shteynbrokkera, divided into four stages.
II degree - a slight fracture of the cartilage and bone, a slight narrowing of the
articular gap, single Uzury bones.
III degree - significant destruction of cartilage and bone, marked narrowing of the
articular gap, multiple Uzury, subluxations, ulnar deviation.
II degree - the deprivation of ability to perform professional activities (in school), and
moderate self-limiting.
III degree - the loss of self-service capabilities and the need for outside care.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis should be made Yura with rheumatoid arthritis, reactive
arthritis, ankylosing spondylarthritis, Reiter's disease. Table 13.6 shows the hallmarks
of these diseases and articular syndrome in particular.
Disease Hallmarks
Rheumatoid Arthritis or arthralgia occur 2-3 weeks after
arthritis
transferred angina
Pain and swelling are held a few days to 2-3 weeks Arthritis pass without
consequences
Reactive Communication arthritis with a specific infection Asymmetric damage of
arthritis joints positive serological tests Effect of antibiotic therapy
HLA-B27
Reiter's disease Communication with chlamydial infection asymmetric arthritis of the lower
extremities unilateral sacroiliac disease reversibility arthritis HLA-B27
Acetylsalicylic acid - 60-80 mg / kg / day, no more than 3 g / day. Assign after meals
3-4 times a day. The duration of 1-3 months depending on individual tolerance.
Diclofenac - 2-3 mg / kg / day, but not more than 100 mg / day in 2 divided doses.
P. Glucocorticoids
Methotrexate appointed interior 2-3 times a week. Usually weekly dose ranged from
2,5 to 7,5 mg / m 2 body surface.
Basic drugs prescribed for long periods from one to several years depending on the
clinical picture of disease. Conduct basic therapy reduces the need for NSAIDs and
glucocorticoids (and, consequently, reduces the risk of side effects occurring during
therapy with these drugs), can improve quality of life, reduce disability, improve long-
term prognosis, and increase longevity.
IV. Immunotherapy
Local therapy
Widely used topical treatment of the affected joint - intraarticular injection of drugs,
mainly corticosteroids, temporary immobilization of the joint with removable splints,
various physiotherapy treatments, exercise therapy, massage. In the presence of
contractures impose skeletal traction, hydrotherapy spend on special equipment.
Complications of therapy
Drugs used to treat Jurassic, have many side effects. Thus, NSAIDs and oral
glucocorticoids in the increase of acid-forming function of the stomach and can cause
a chronic hyper-atsidny gastritis with the development of erosive and ulcerative
process, so they must be taken after meals, preferably an alkaline drink drink. If a
child is suffering from Jurassic and receiving treatment, complaining of abdominal
pain, an urgent need to show it to your doctor to endoscopic examination of the
stomach, not to miss a serious complication such as perforation of ulcers.
PREVENTION
Prevention can only be secondary, ie relapse prevention Yura, and even then only if
the patient is constantly monitored by rheumatologists. Secondary prevention involves
constant monitoring of clinical manifestations and laboratory parameters of disease. In
the presence of incipient acute need to strengthen drug therapy, to reduce physical
exertion (training at home for school) and, if necessary, put the patient in a hospital,
preferably one where he previously was.
FORECAST
Jura - a disease for life, but when correctly chosen therapy and systematic observation
rheumatologist possible long-term remission with a satisfactory quality of life (access
to education, the acquisition of secondary and higher education, the work of the
profession). If you often recurrent course, manifestations of systemic disease more
pessimistic prognosis - early going disability, active life is limited.