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JUVENILE RHEUMATOID ARTHRITIS

Juvenile rheumatoid arthritis (Jura) - a chronic inflammatory joint disease in children


younger than 16 years with unknown etiology and complex pathogenesis,
characterized by steadily progressive course and is accompanied in some patients the
involvement of internal organs, often ending with a disability.

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.

Acute onset is usually characteristic of the severe form --


generalized articular or articular-visceral (systemic) form of the disease with
frequently recurrent course and a poor prognosis. This form is more common in
children of preschool and primary school age, but it may also occur in adolescents.

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-visceral (systemic form) includes five features: persistent high fever,


polymorphic allergic rash, lymphadenopathy, gepatolienalny syndrome, arthralgia /
arthritis.This form Yura has two main options (Table 13-3) - Still syndrome, usually
develops in children of preschool age, and Wissler-Fanconi's syndrome, usually
observed in school children.

Table 13-3. Options system forms Juvenile rheumatoid arthritis

Major clinical symptoms Syndrome Syndrome


Stnlla Wissler-Fanconi
Acute onset + +
Moderate fever + --
The high persistent fever -- +
Polyarthritis with lesions of small joints + --
Polyarthritis with the defeat of large joints, -- +
including hip
Long arthralgia +
--
Lymphadenopathy + +
Hepatosplenomegaly + +
Poliserozit + +
Myocarditis + +
Anemia + --
Neutrophilic hyperskeocytosis +
--
Sharp increase in ESR + +

Long-term recurring during Jurassic may be complicated secondary amyloidosis,


helped by the constant circulation in the bloodstream of immune complexes. Amyloid
is deposited in the walls of blood vessels in the kidneys, liver, myocardium, intestines,
which leads to disruption of their functions. Most amyloidosis affects the kidneys, as
evidenced by Nuria-resistant to the development of subsequent renal failure.

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

1. Arthritis duration of 3 months or more.

2. Arthritis is the second joint, which emerged after 3 months later.


3. Symmetrical lesion of small joints.

4. Contractures of joints.

5. Peritendinitis or bursitis.

6. Muscle atrophy.

7. Morning stiffness.

8. Rheumatoid eye disease.

9. Rheumatoid nodules.

10. Effusion in the cavity of joints. Radiographic signs

11. Osteoporosis, melkokistoznaya alteration of bone structure epiphysis.

12. Narrowing of joint cracks, bone erosion, ankylosis of the joints (Figure 13.6 inset).

13. Violation of bone growth.

14. The defeat of the cervical spine. Laboratory signs

15. Positive rheumatoid factor.

16. Positive data biopsy of the synovial membrane.

Depending on the number of identified positive attributes determine the degree of


probability of disease (with mandatory presence of arthritis):

3 trait - probable Jurassic;

4 features - some Jurassic;

8 characters - a classic Jurassic.

Table 13-4. Working Classification of juvenile rheumatoid arthritis

Shape Flow Activity, The presence of rheumatoid Functional


degree factor in the blood violations, the degree
- Predominantly articular (mono - - Quickly I Rheumatoid factor I
arthritis, oligoarthritis, polyarthritis) progressive II "+" II
- System (with cardiac, - Moderately Rheumatoid factor III
nH
reticuloendothelial system progressive "-"
light, with vasculitis, poliserozitom) - Slowly
- Still Syndrome progressive
- Syndrome of Wissler-Fanconi
(allergoseptichesky syndrome)
- The joint form with defeat
eye - rheumatoid uveitis

Table 13-5. Anglo-American classification of juvenile rheumatoid arthritis

I. System Form

II. Poliarticular form

Subtype with the presence of rheumatoid factor

Subtype of the lack of rheumatoid factor

III. Oligoartikulyarnaya 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.

I degree - osteoporosis without destructive changes.

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.

IV degree - the degree of symptoms III in combination with ankylosis.

As a result of rheumatoid inflammation evolving changes in the joints with three


degrees, depending on the nature and gravity of the violation of their functions. The
degrees of dysfunction of the joints as follows.

I degree - a moderate restriction of professional activities (study


school), but full of self-preservation.

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.

Table 13-6. Differential Diagnosis Juvenile rheumatoid arthritis *

Disease Hallmarks
Rheumatoid Arthritis or arthralgia occur 2-3 weeks after
arthritis
transferred angina

The joints are affected symmetrically

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

Bechterew Early bilateral sacroiliac disease

Slowly progressive mono-, oligoarthritis

Often infected hip

Exostoses heel bones

HLA-B27
Reiter's disease Communication with chlamydial infection asymmetric arthritis of the lower
extremities unilateral sacroiliac disease reversibility arthritis HLA-B27

* From: Nasonova VA et al. Clinical Rheumatology. - M., 1989


TREATMENT

Treatment of Jurassic to conduct a comprehensive and phased manner. In the active


period of patients in need of hospital treatment in the inactive - in outpatient
observation and spa treatment. Much of the time, patients are treated on an outpatient
basis in connection with the duration of the disease. In the clinic, children continue to
receive combined-tion therapy, drug therapy, exercise therapy, massage and
physiotherapy courses. Only long-term and continuous treatment under the
supervision of doctors and nurses can have a positive effect .

In the period of acute treatment includes NSAIDs, in severe cases, in combination


with glucocorticoids and immunosuppressants (quinoline derivatives, penicillamine,
methotrexate, cyclosporine), as well as with normal human Ig. Below are the main
drugs, their doses and duration of application.

The main drugs used in the treatment of juvenile rheumatoid arthritis

I. Non-steroidal anti-inflammatory drugs

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.

Indometacin - 2-3 mg / kg / day. Young children prescribed 25 mg / day (1 / 2


tablets 2 times a day). Older children designate up to 100 mg / day (2 tablets of 50 mg
in 2 divided doses).

Diclofenac - 2-3 mg / kg / day, but not more than 100 mg / day in 2 divided doses.

Ibuprofen - 200-1000 mg / day depending on age at the rate of 40 mg / kg / day in 3


divided doses.

Naproxen - 250-750 mg / day depending on age. Not recommended for children


under 10 years. Duration of the course of several months to several years.

P. Glucocorticoids

Prednisolone - into a cost of 1 mg / kg / day (maximum dose). Duration of the course


- on the testimony.

Methylprednisolone (metipred), betamethasone (diprospan) - vnutrisustav-no. The


dose depends on the joint. In the same joint drug impose no more than 5 times at
intervals of 5 days. The course may be repeated.
III. Basic drugs

Quinoline: hydroxychloroquine (Plaquenil) and chloroquine (hingaminom, de lagil).


Hydroxychloroquine in a dose of 200-300 mg 1 time daily, preferably before going to
sleep after eating. Chloroquine at a dose of 125-250 mg / day depending on age, 1
time per day at night after eating.

Methotrexate appointed interior 2-3 times a week. Usually weekly dose ranged from
2,5 to 7,5 mg / m 2 body surface.

Sulfasalazine appoint 0,5-1 g / day in 2 divided doses.

Penicillamine appointed interior of 60-125 mg in 1 reception for 1-2 hours before


breakfast for 1,5-2 months.

Cyclosporine prescribe a dose of 2-3 mg / kg / day in 2 hours under the control of


the level of serum creatinine. If you increase it by more than 30% dose reduced or
abolished. The course of treatment 2 months or more may be needed.

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

Ig for intravenous administration (eg, pentaglobin, intraglobin, sandoglobulin) at a


dose of 0,4-2 g / kg / day for 4-5 days. Enter the drops, but at 10-20 drops per minute
for 15 minutes, then increase speed to 2 ml / min. If necessary, repeat infusions every
4 weeks.

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.

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