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НАО МУС
Juvenile idiopathic arthritis– a formidable
chronic disease of childhood, affecting the
joints, requiring timely diagnosis, treatment
and premature prevention.
normal jointl arthritis
Etiology
Viral and bacterial infection (Coxsackie
group enteroviruses, rubella, hepatitis viruses,
adenoviruses)
Heredity
Medications
Injuries
Hypothermia and excessive insolation
The pathogenesis of
juvenile idiopathic
arthritis
The etiological factor acts in two
directions:
The etiological factor is
localized on the synovial
membrane of the joint.
Local immune reaction with
the formation of altered IgGs
with autoreactivity.
Aggregated IgG are
recognized as foreign
antigens.
The synovial membrane of the
joint swells, hyperemia, the
amount of synovial fluid increases.
Therefore, synovitis occurs as a result of a local immune-inflammatory
reaction.
1.
2. Antigenic determinants (viral particles, peptidoglycan of
the bacterial wall, etc.) of the etiological factor
Activation of 1a-antigen-
positive cells
Isolation of interleukin-1
Activation of T-lymphocytes-
helpers
Activated helper T-lymphocytes
secrete lymphokines and
interleukins.
Intensive synthesis of interleukin-
2.
It binds to receptors on the surface
of activated T cells and stimulates
their proliferation.
Increased synthesis of T-cell
cytokines
(interleukin-6, T-nuclear factor,
etc.).
Proliferation of B-lymphocytes
and their transformation into
plasma cells.
Synthesis of antibodies.
Perceiving the altered IgG as a foreign antigen,
plasma cells produce antibodies of the IgG and IgM
classes.
These are the so-called rheumatoid factors, i.e.
autoantibodies.
Rheumatoid factors - leukotrienes, IgM, or to the
Fc1 fragment of an altered IgG.
Components C3a and
C5a of the
complement system
Chemotaxis of neutrophils,
macrophages and lymphocytes in the
pathological focus
Synovial membrane infiltration, hypertrophy, inflammatory
granulation tissue - pannus.
Pannus is a granulation tissue consisting of
proliferating fibroblasts, lymphoid cells, macrophages
and is rich in blood vessels.
Primary alteration leads to activation of the
Hageman factor
kinin system blood clotting
Violation of
microcirculation
Microthrombosis
Changes in small vessels
Biologically active substances
(serotonin, prostaglandins, kinins,
etc.)
Activates
Macrophage
activation
Isolation of
monokines
Phagocytosis of
immune
complexes by
neutrophils
Formation of
ragocytes
Macrophage
Phagocytosis
Destruction of
phagocytes
Inflammatory mediators
Prostaglandins, vasoactive
amines, proteases, leukotrienes
and oxygen radicals
Synovial cells produce collagenase,
leading to cartilage degradation.
Prostaglandins stimulate the activity of
osteoclasts and increase the resorption
of calcium from bones, leading to
erosions of the articular bone.
Pannus crawls onto the articular
surface
Penetrates into articular cartilage
Dystrophic and destructive changes in
the form of cracks, erosion,
osteoporosis
Loss of cartilage, replaced by
granulation tissue.
fibrosis and
sclerosis
capsules
the formation of
fibrous adhesions
Etched appearance
of the epiphyses or
complete destruction
Bone
ankylosis
Muscles around affected joints
atrophy
Rheumatoid subcutaneous
nodules, (lymphatic granulomas,
with areas of fibrinoid necrosis
surrounded by histiocytes and
lymphocytes)
CLINICAL AND ANATOMICAL
CHARACTERISTICS OF THE DISEASE:
Mainly articular form:
 Polyarthritis;
 Oligoarthritis (2-3 joints);
 Monoarthritis.
Articular-visceral form :
 With limited visceritis;
 Still's syndrome;
 Allergoseptic syndrome (Wissler-Fanconi).
Rheumatoid arthritis with eye involvement.
CLINICAL AND IMMUNOLOGICAL
CHARACTERISTICS:
 JRA - RF "+"
 JRA - RF "-"
 Rapidly progressing;
 Slowly progressing.
THE DEGREE OF ACTIVITY OF THE
PROCESS:
I
II Degrees
III
Remissions
X-RAY STAGE OF ARTHRITIS :
I - periarticular osteoporosis; signs of effusion in the
joint cavity; compaction of periarticular tissue;
II - osteoporosis, cartilage destruction, narrowing of the
joint space; single bone usuras;
III - bone erosion; pronounced bone and cartilage
destruction; dislocations; subluxations; systemic
dysplasia of bones;
IV - ankylosis.
FUNCTIONAL ABILITY OF THE
PATIENT:
I – the ability to self-service is preserved;
II – the ability to self-service is partially preserved;
III – the ability to self-service is completely lost.
The onset of the disease may be acute
or subacute.
With an acute onset, body temperature
rises, soreness appears, and then
swelling in one or more joints, often
symmetrical small joints.
The symmetry of the lesions becomes
apparent not immediately, but within a
few days or weeks from the onset of
the disease.
All joints can be affected, but
large joints - knee, ankle, wrist
- are more often affected.
Smaller joints of the hands
and feet (metatarsophalangeal,
interphalangeal) suffer less
frequently.
Typical for juvenile
rheumatoid arthritis is the
defeat of the joints of the
cervical spine.
Acute onset
 characteristic of severe forms - a generalized articular or
articular-visceral (systemic) form of the disease with a
relapsing course and an unfavorable prognosis.
 This form is more often observed in children of
preschool and primary school age, but it can also occur
in adolescents.
It starts with one joint - the knee or ankle.
The joint swells, its function is disturbed.
The child's gait changes, and children under 2 years of age stop walking.
Accompanied by rheumatoid uveitis, unilateral or bilateral.
In which all the membranes of the eye are affected, as a result of which visual acuity drops
sharply up to its complete loss.
Oligoarticular form - a form
of the disease that occurs
with the involvement of
several joints in the process
(2-4)
Pain in the joints is moderate, as
are exudative changes.
The process may involve two
ankles, one knee joint, and vice
versa.
Body temperature
does not rise.
Moderate
polyadenitis.
It proceeds benignly, with
less frequent exacerbations.
The articular-visceral form includes
five features:
Persistent high fever
Polymorphic allergic rash
Lymphadenopathy
Hepatolienal syndrome
Arthralgia/arthritis
Still's syndrome
High hectic fever, generalized enlargement of
lymph nodes, liver, spleen.
Wissler-Fanconi
syndrome
The articular-visceral form has two main
options:
In some patients, damage to internal organs:
heart, kidneys, lungs.
Most children have a varied rash
Severe articular syndrome, difficult to treat
and destruction of bone tissue
Hyperleukocytosis, neutrophilia with a shift to
the left, a sharply increased ESR
Begins acutely with high fever (mainly at
night), polymorphic rash (macular,
urticarial, acular)
Articular syndrome
Visceral manifestations
In half of the patients, subsepsis is acute
and ends in recovery after one or two acute
periods.
In other children, the disease acquires the
features of JCA.
In preschool children
More often in
schoolchildren
Laboratory diagnostics of JRA
-general and biochemical blood tests;
- test for procalcitonin;
- blood and urine cultures for sterility;
- smear from the throat and nose for microflora;
-coagulogram;
- puncture of the bone marrow;
-immunological indicators;
- serological studies for the detection
- Salmonella, Shigella, Yersinia, Brucella, Chlamydia, Toxoplasma, Toxocar, Trichinella, as
well as respiratory, herpetic viruses, Epstein-Barr virus, hepatitis B and C viruses, CMV,
HIV.
Laboratory tests may show the following changes:
• in the general blood test: a sharp acceleration of ESR (up to 50-80 mm / h);
increase in the number of leukocytes (especially with allergic septic
syndrome) with an increase in stab neutrophils, anemia (with a long course
of the disease), thrombocytosis;
• biochemical blood test: increase in the content of seromucoid,
diphenylamine reaction;
• immunological studies: an increase in C-reactive protein, an increase in the
content of immunoglobulins of classes M and G in the blood, a decrease in
complement levels, the detection of rheumatoid factor, sometimes a
positive ANF;
• special research methods: detection of HLADR4 antigen, HLA A2, HLA
B27.
The degree of activity of juvenile rheumatoid
arthritis can be determined by laboratory
parameters:
• Grade 0: ESR up to 12 mm/h, C-reactive protein is normal;
• I degree: ESR 13-20 mm/h, C-reactive protein is slightly elevated ("+");
• II degree: ESR 21-39 mm/h, C-reactive protein is moderately elevated
("++");
• III degree: ESR 40 mm/h or more, C-reactive protein is sharply increased
("+++", "++++").
Instrumental diagnostic methods:
1) X-ray of the affected joints. According to the data obtained, the
following stages of the pathological process are distinguished:
• Stage I: osteoporosis in the area of the epiphyses.
• Stage II: cartilage is defibrillated, the joint space narrows, single erosions
appear.
• Stage III: destruction of cartilage and bone tissue occurs, bone-
cartilaginous erosions, subluxations in the joints are formed.
• Stage IV: stage III criteria + fibrous or bone ankylosis.
2) ECG and echocardiography to detect myopericarditis. On the ECG, signs
of overload of the heart departments, violations of the processes of
myocardial repolarization can be recorded. According to EchoCG: an
increase in the cavity of the left ventricle, a decrease in ejection fraction,
hypokinesia of the posterior wall of the left ventricle and / or interventricular
septum, signs of relative insufficiency of the mitral and / or tricuspid valves,
increased pressure in the pulmonary artery, separation of the sheets of the
pericardium and / or the presence of free fluid in pericardial cavities.
3) X-ray of the chest organs: with damage to the heart - an increase in the size of the
shadow of the heart, an increase in the pulmonary pattern and focal shadows. When
the lungs are damaged, there may be an increase and deformation of the lung pattern,
heavy seals and cellular enlightenments (a picture of a “honeycomb” lung). X-ray
examination of the joints. In the first months of the disease, the main indicator is
epiphyseal osteoporosis. Then erosion appears.
4) In difficult cases, for the diagnosis of articular syndrome, computed tomography
and magnetic resonance imaging are used to accurately assess the development of
pannus, articular effusion, cartilage loss and meniscal hypotrophy.
Tomography of the joints - gamma scintigraphy with technetium-99 determines local
minimal changes.
The severity of radiographic changes on the
Steinbroker scale:
Stage I - osteoporosis, mainly epiphyseal.
Stage II - osteoporosis and initial cartilage destruction,
narrowing of the joint space.
Stage III - pronounced destruction of cartilage and bone, bone
erosion.
Stage IV - symptoms of stage III and ankylosis.
TREATMENT
• therapy begins
immediately after the
diagnosis of JRA;
• the aggressiveness of
immunosuppressive therapy
should correspond to the
aggressiveness of the
course of the disease.
GOALS OF JRA
THERAPY:
• Suppression of the inflammatory and
immunological activity of the process;
• Relief of systemic manifestations and articular
syndrome;
• Preservation of the functional ability of the
joints;
• Prevention or slowdown of joint destruction,
disability of patients;
• Achieving remission;
• Improving the quality of life of patients;
• Minimize the side effects of therapy.
NON-DRUG THERAPY
1.Mode
During the period of exacerbation, the
motor mode is limited.
Complete immobilization is
contraindicated, as it contributes to the
development of contractures,
osteoporosis, ankylosis, and physical
exercises help to maintain the functional
activity of the joints.
Useful: cycling, swimming, walking.
Undesirable: running, jumping, active
games.
Exclude exposure to the sun and psycho-
emotional overload.
2. Therapeutic exercise is the most important component of JRA
treatment.
Daily exercises are needed to increase the range of motion in the
joints, eliminate flexion contractures, and restore muscle mass.
In case of damage to the hip joints, traction procedures are
prescribed for the affected limb, walking on crutches.
During the development of coxitis and aseptic necrosis of the
femoral heads, the movement of the patient without crutches is
contraindicated.
Physiotherapy exercises should be carried out in accordance with
the individual capabilities of the patient.
3. Orthopedic correction
Static orthoses such as splints, splints, insoles are
used - they should be worn or put on in their free
time and must be removed during the day to
stimulate the muscular system during exercise,
classes, occupational therapy.
In case of severe osteoporosis in the cervical spine,
it is mandatory to wear a head holder (soft, hard),
and in case of osteoporosis of the thoracic and
lumbar spine, it is necessary to wear a corset or
reclining system.
MEDICAL THERAPY
1. Symptom Modifying Therapy
(anti-inflammatory, symptomatic):
1.1. Non-steroidal anti-inflammatory drugs.
1.2. GCS: local therapy (intra-articular), pulse therapy.
2. Disease modifying therapy
(pathogenetic, basic)
2.1. Cytotoxic drugs: methotrexate, cyclosporine A,
leflunomide.
3. Biological agents: infliximab, rituximab.
1.1. Non-steroidal anti-inflammatory drugs (NSAIDs).
RATIONAL APPROACH TO NSAID
THERAPY:
• Reception mode: start treatment with the lowest dose,
with good tolerance after 2-3 days, the dose can be
increased, take after meals, after taking NSAIDs, do
not lie down for 15 minutes in order to prevent
esophagitis.
• Refusal of the simultaneous use of 2 or more
NSAIDs.
• Preventive treatment of NSAID gastropathy - acid-
regulating drugs: proton pump inhibitors
(omeprazole), H2-blockers (famotidine, ranitidine)
International title Tradename Dose
mg/kg/day
Multiplicity
of reception
Application, with
age
Diclofenac Voltaren
Diclofenac
Naklofen
Ortofen
2-3 2-3 5 years
Naproxen Naproxen
Apranax Naprios
15-20 2 5 years
Ibuprofen Ibuprofen
Ibufen
Nurofen
35-40 2-4 3 months
Piroxicam Pyroxifer
Feldoral
0,3-0,6 1-2 12 years
Selective COX-2 inhibitors
Nimesulide Nise, Nimulid 3-5 2-3 up to 6 years with
caution
Meloxicam Movalis,
Meloxicam
0,3-0,5 1 15 years
NSAIDs used to treat arthritis in children:
• 1.2. Glucocorticosteroids (GCS)
• Prednisolone
Dose:
• High doses — 0.6-1.0 mg/kg/day
• Average doses - 0.3-0.6 mg / day
• Low doses — 0.3-0.1 mg/kg/day
The initial dose is 0.2-0.5 mg/kg/day (15 mg/day) for no more than
1 month until remission is achieved, then the dose is reduced to a
maintenance dose (0.1-0.15 mg/kg) followed by withdrawal.
1.2.1. PULSE THERAPY GCS
Indications:
• Severe systemic manifestations in
JRA:
- myopericarditis;
- pneumonitis, pleuropneumonitis;
- polyserositis, vasculitis
• JRA complications:
- cardiopulmonary insufficiency;
- macrophage activation syndrome;
• Ineffectiveness of previous therapy.
Pulse therapy mode:
Methylprednisolone 5-10 mg/kg/administration
(no more than 500 mg) in 200 ml of saline for
35-40 minutes for 2-5 days in a row.
In case of inefficiency:
- increase the duration of the GC pulse therapy
— repeat the course of GC pulse therapy
1.2.2. LOCAL THERAPY OF
CORTICOSTEROIDS (INTRA-ARTICULAR)
• Indications:
• Mono-, oligoarthritis of moderate or high local activity;
• Predominant defeat of 1 or 2 joints in polyarthritis;
• With JRA at the beginning of basic therapy with high
local activity of 1-2 joints;
• If there are contraindications for basic therapy as a
temporary palliative method;
• Prevention of deformity as a component of a
rehabilitation program.
CONTRAINDICATIONS:
• local or systemic infection
• Pronounced bone destruction
• Severe periarticular osteoporosis
• Difficult access to the joint
• Pathology of the blood coagulation
system
• Ineffectiveness of previous local GC
therapy
The amount of GCS for intra-articular
administration
Joint The amount of the
drug
Large knee,
ankle,
shoulder
1-2 ml
Medium Elbow, wrist 0,5-1 ml
Small interphalangeal,
metacarpophalangeal,
metatarsophalangeal
0,1-0,5 ml
Enter no more than 1 time per month
2. CYTOTOXIC THERAPY
2.1. Methotrexate
Rational principles of methotrexate therapy
Appointment in the early stages of the disease.
Application for a long period.
Correction of therapy in case of ineffectiveness with
adequate terms of treatment.
Prevention of side effects of methotrexate - the
appointment of folic acid - 5 mg / week.
The standard dose of methotrexate is 10 mg/m2/week
(0.3-0.5 mg/kg)
The calculation of the body area is made according to
nomographic tables or the Kosteff formula:
S = 4m+7 , where m is the patient's body weight
RULES FOR PRESCRIBING
CYCLOSPORINE
• Appointment in a specialized hospital.
• Double control of serum creatinine
concentration before prescribing the drug.
• Cancel NSAIDs or reduce the daily dose in
half.
• Do not use with drugs that enhance the
nephrotoxicity of cyclosporine:
- macrolides
- aminoglycosides.
• Initial dose — 3.5 mg/kg/day.
• • The daily dosage is divided into 2 doses of 1.5 mg/kg every
12 hours.
• • The effect develops in 1-3 months and reaches a maximum
within 6-12 months.
• • Increasing the dose is carried out with the ineffectiveness of
the initial dose after 2 months of treatment, not more than 1
time in 14 days, 25 mg.
• • Do not exceed a daily dose of 5 mg/kg.
• Monitoring treatment with cyclosporine
• • Physical examination - once a month
• • Clinical and biochemical blood tests - once every 2 weeks -
control of serum concentrations: - creatinine, - urea, - uric acid,
- aminotransferases (ALT, AST), - bilirubin, - potassium;
• • Consultation with an ophthalmologist, examination with a slit
lamp for all children with JRA — 1 time in 3 months.
• • Measurement of blood pressure every day.
• 2.3. Leflunomide (Arava) is indicated for severe JRA,
torpid to classical immunosuppressants.
• Mechanism of action of leflunomide
• • Leflunomide - acts as an immunomodulator (without
a cytotoxic effect), since it has much less effect on
other cells (when other cells divide, the accumulated
reserves of pyrimidine precursors are used).
• Additional mechanisms:
• - Inhibits the activation of NF-kB (a mediator of pro-
inflammatory genes).
• - Blocks the expression of cell adhesion molecules.
• - Reduces the production of free radicals.
• - Influence on COX.
3. BIOLOGICAL AGENTS
3.1. Infliximab (Remicade) is a monoclonal antibody
against TNF-a.
Dose: 3-6 mg/kg per regimen: 0, 2, 6 and then every 8
weeks.
The use of leflunomide in pediatric practice: it is most
effective in the early stages of JRA in patients with oligo-
and polyarticular variants. It can be used in children over
10 years of age in a highly specialized rheumatology
hospital with the permission of the ethical committee and
the informed consent of the parents.
3.2. Rituximab (MabThera)
is a monoclonal antibody to
CD20 receptors on the
surface of B-lymphocytes.
Dose: 375 mg/km2 per
administration schedule: 0,
1, 2.3 weeks.
Use in pediatric practice: in
severe systemic JRA
variants refractory to
standard therapy.
COMBINED IMMUNOSUPPRESSIVE
THERAPY JRA
3 main types of combination therapy:
• Initiation of treatment with one drug and the
appointment of several immunosuppressants after
8-12 weeks while maintaining disease activity.
• Start of treatment with combination therapy with
transfer to monotherapy (after 3-12 months) with
suppression of the activity of the process.
• Carrying out combination therapy during the
entire period of the disease.
COMBINATION THERAPY OPTIONS:
1. Combined pulse therapy with high doses of methotrexate and methylprednisolone
· Methylprednisolone 15-20 mg/kg for 3 consecutive days.
Methotrexate - EBEWE 30 mg/m2 in 2nd infusion followed by 15 mg/m2 IM weekly
and repeated quarterly pulse therapy.
2. Combined therapy with methotrexate and cyclosporine.
Methotrexate - 12-15 mg / m2 / week orally or initially pulse therapy at a dose of 50 mg
/ m2 / week for 8 weeks, switching to an intramuscular injection at a dose of 15 mg /
m2 / week.
Cyclosporine A - 3.5-5 mg / kg / day.
3. Combination therapy with leflunomide and methotrexate.
Methotrexate - 10-12 mg / m2 / week for 8 weeks.
Leflunomide: patients weighing <30 kg - 50 mg/day for 3 consecutive days, then 10
mg/day; patients weighing >30 kg — 100 mg/day for 3 consecutive days, then 20
mg/day.
DISPENSARY OBSERVATION
• Observation in an outpatient setting is carried out by a pediatric
rheumatologist, a pediatric cardiorheumatologist or a
pediatrician who has completed an advanced training cycle in
pediatric rheumatology, taking into account the
recommendations of a specialized rheumatology department.
• Physical examination is carried out once a month.
• When treating with immunosuppressants, clinical and
biochemical blood tests are performed once every 2 weeks
(protein and fractions, urea, creatinine, bilirubin. ALT, AST,
alkaline phosphatase, calcium, potassium, sodium).
• · Analysis of immunological parameters is carried out 1 time in
3 months (IgG, IgM, IgA, CRP, RF, ANF).
• ECG once every 3 months.
JRA FORECAST
• • 40-50% of children have a
favorable prognosis
• • 1/3 of patients have a disease;
continuously recurs;
• • 20% of patients develop severe
destructive arthritis;
• • 20% develop amyloidosis in
adulthood;
• • 65% have severe functional
impairment.
FORECAST OF JRA SYSTEM
VARIANTS:
JRA COMPLICATIONS
1. Amyloidosis - due to the
constant circulation of
immune complexes in the
bloodstream. Amyloid is
deposited in the walls of
blood vessels, in the kidneys,
liver, myocardium, and
intestines, which leads to
disruption of their functions.
2. Macrophage activation
syndrome (MAS).
Trigger factors: bacterial, viral
(CMVI, HSV, etc.) infection,
drugs (NSAIDs, gold salts) In
JA, the clinical picture of CAM
may resemble sepsis or
exacerbation of the underlying
disease: persistent fever,
hepatosplenomegaly,
cytopenia.

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JIA.pptx

  • 2. Juvenile idiopathic arthritis– a formidable chronic disease of childhood, affecting the joints, requiring timely diagnosis, treatment and premature prevention. normal jointl arthritis
  • 4. Viral and bacterial infection (Coxsackie group enteroviruses, rubella, hepatitis viruses, adenoviruses)
  • 9. The pathogenesis of juvenile idiopathic arthritis
  • 10. The etiological factor acts in two directions: The etiological factor is localized on the synovial membrane of the joint. Local immune reaction with the formation of altered IgGs with autoreactivity. Aggregated IgG are recognized as foreign antigens. The synovial membrane of the joint swells, hyperemia, the amount of synovial fluid increases. Therefore, synovitis occurs as a result of a local immune-inflammatory reaction. 1.
  • 11. 2. Antigenic determinants (viral particles, peptidoglycan of the bacterial wall, etc.) of the etiological factor Activation of 1a-antigen- positive cells Isolation of interleukin-1 Activation of T-lymphocytes- helpers
  • 12. Activated helper T-lymphocytes secrete lymphokines and interleukins. Intensive synthesis of interleukin- 2. It binds to receptors on the surface of activated T cells and stimulates their proliferation. Increased synthesis of T-cell cytokines (interleukin-6, T-nuclear factor, etc.). Proliferation of B-lymphocytes and their transformation into plasma cells. Synthesis of antibodies.
  • 13. Perceiving the altered IgG as a foreign antigen, plasma cells produce antibodies of the IgG and IgM classes. These are the so-called rheumatoid factors, i.e. autoantibodies. Rheumatoid factors - leukotrienes, IgM, or to the Fc1 fragment of an altered IgG.
  • 14. Components C3a and C5a of the complement system Chemotaxis of neutrophils, macrophages and lymphocytes in the pathological focus Synovial membrane infiltration, hypertrophy, inflammatory granulation tissue - pannus. Pannus is a granulation tissue consisting of proliferating fibroblasts, lymphoid cells, macrophages and is rich in blood vessels.
  • 15. Primary alteration leads to activation of the Hageman factor kinin system blood clotting Violation of microcirculation Microthrombosis Changes in small vessels Biologically active substances (serotonin, prostaglandins, kinins, etc.) Activates
  • 16. Macrophage activation Isolation of monokines Phagocytosis of immune complexes by neutrophils Formation of ragocytes Macrophage Phagocytosis
  • 17. Destruction of phagocytes Inflammatory mediators Prostaglandins, vasoactive amines, proteases, leukotrienes and oxygen radicals
  • 18. Synovial cells produce collagenase, leading to cartilage degradation. Prostaglandins stimulate the activity of osteoclasts and increase the resorption of calcium from bones, leading to erosions of the articular bone.
  • 19. Pannus crawls onto the articular surface Penetrates into articular cartilage Dystrophic and destructive changes in the form of cracks, erosion, osteoporosis Loss of cartilage, replaced by granulation tissue.
  • 20. fibrosis and sclerosis capsules the formation of fibrous adhesions Etched appearance of the epiphyses or complete destruction Bone ankylosis
  • 21. Muscles around affected joints atrophy Rheumatoid subcutaneous nodules, (lymphatic granulomas, with areas of fibrinoid necrosis surrounded by histiocytes and lymphocytes)
  • 22.
  • 23.
  • 24. CLINICAL AND ANATOMICAL CHARACTERISTICS OF THE DISEASE: Mainly articular form:  Polyarthritis;  Oligoarthritis (2-3 joints);  Monoarthritis. Articular-visceral form :  With limited visceritis;  Still's syndrome;  Allergoseptic syndrome (Wissler-Fanconi). Rheumatoid arthritis with eye involvement.
  • 25. CLINICAL AND IMMUNOLOGICAL CHARACTERISTICS:  JRA - RF "+"  JRA - RF "-"
  • 26.  Rapidly progressing;  Slowly progressing.
  • 27. THE DEGREE OF ACTIVITY OF THE PROCESS: I II Degrees III Remissions
  • 28. X-RAY STAGE OF ARTHRITIS : I - periarticular osteoporosis; signs of effusion in the joint cavity; compaction of periarticular tissue; II - osteoporosis, cartilage destruction, narrowing of the joint space; single bone usuras; III - bone erosion; pronounced bone and cartilage destruction; dislocations; subluxations; systemic dysplasia of bones; IV - ankylosis.
  • 29. FUNCTIONAL ABILITY OF THE PATIENT: I – the ability to self-service is preserved; II – the ability to self-service is partially preserved; III – the ability to self-service is completely lost.
  • 30. The onset of the disease may be acute or subacute. With an acute onset, body temperature rises, soreness appears, and then swelling in one or more joints, often symmetrical small joints. The symmetry of the lesions becomes apparent not immediately, but within a few days or weeks from the onset of the disease.
  • 31. All joints can be affected, but large joints - knee, ankle, wrist - are more often affected. Smaller joints of the hands and feet (metatarsophalangeal, interphalangeal) suffer less frequently. Typical for juvenile rheumatoid arthritis is the defeat of the joints of the cervical spine.
  • 32. Acute onset  characteristic of severe forms - a generalized articular or articular-visceral (systemic) form of the disease with a relapsing course and an unfavorable prognosis.  This form is more often observed in children of preschool and primary school age, but it can also occur in adolescents.
  • 33. It starts with one joint - the knee or ankle. The joint swells, its function is disturbed. The child's gait changes, and children under 2 years of age stop walking. Accompanied by rheumatoid uveitis, unilateral or bilateral. In which all the membranes of the eye are affected, as a result of which visual acuity drops sharply up to its complete loss.
  • 34.
  • 35. Oligoarticular form - a form of the disease that occurs with the involvement of several joints in the process (2-4) Pain in the joints is moderate, as are exudative changes. The process may involve two ankles, one knee joint, and vice versa. Body temperature does not rise. Moderate polyadenitis. It proceeds benignly, with less frequent exacerbations.
  • 36. The articular-visceral form includes five features: Persistent high fever Polymorphic allergic rash Lymphadenopathy Hepatolienal syndrome Arthralgia/arthritis
  • 37. Still's syndrome High hectic fever, generalized enlargement of lymph nodes, liver, spleen. Wissler-Fanconi syndrome The articular-visceral form has two main options: In some patients, damage to internal organs: heart, kidneys, lungs. Most children have a varied rash Severe articular syndrome, difficult to treat and destruction of bone tissue Hyperleukocytosis, neutrophilia with a shift to the left, a sharply increased ESR Begins acutely with high fever (mainly at night), polymorphic rash (macular, urticarial, acular) Articular syndrome Visceral manifestations In half of the patients, subsepsis is acute and ends in recovery after one or two acute periods. In other children, the disease acquires the features of JCA. In preschool children More often in schoolchildren
  • 38. Laboratory diagnostics of JRA -general and biochemical blood tests; - test for procalcitonin; - blood and urine cultures for sterility; - smear from the throat and nose for microflora; -coagulogram; - puncture of the bone marrow; -immunological indicators; - serological studies for the detection - Salmonella, Shigella, Yersinia, Brucella, Chlamydia, Toxoplasma, Toxocar, Trichinella, as well as respiratory, herpetic viruses, Epstein-Barr virus, hepatitis B and C viruses, CMV, HIV.
  • 39. Laboratory tests may show the following changes: • in the general blood test: a sharp acceleration of ESR (up to 50-80 mm / h); increase in the number of leukocytes (especially with allergic septic syndrome) with an increase in stab neutrophils, anemia (with a long course of the disease), thrombocytosis; • biochemical blood test: increase in the content of seromucoid, diphenylamine reaction; • immunological studies: an increase in C-reactive protein, an increase in the content of immunoglobulins of classes M and G in the blood, a decrease in complement levels, the detection of rheumatoid factor, sometimes a positive ANF; • special research methods: detection of HLADR4 antigen, HLA A2, HLA B27.
  • 40. The degree of activity of juvenile rheumatoid arthritis can be determined by laboratory parameters: • Grade 0: ESR up to 12 mm/h, C-reactive protein is normal; • I degree: ESR 13-20 mm/h, C-reactive protein is slightly elevated ("+"); • II degree: ESR 21-39 mm/h, C-reactive protein is moderately elevated ("++"); • III degree: ESR 40 mm/h or more, C-reactive protein is sharply increased ("+++", "++++").
  • 41. Instrumental diagnostic methods: 1) X-ray of the affected joints. According to the data obtained, the following stages of the pathological process are distinguished: • Stage I: osteoporosis in the area of the epiphyses. • Stage II: cartilage is defibrillated, the joint space narrows, single erosions appear. • Stage III: destruction of cartilage and bone tissue occurs, bone- cartilaginous erosions, subluxations in the joints are formed. • Stage IV: stage III criteria + fibrous or bone ankylosis.
  • 42. 2) ECG and echocardiography to detect myopericarditis. On the ECG, signs of overload of the heart departments, violations of the processes of myocardial repolarization can be recorded. According to EchoCG: an increase in the cavity of the left ventricle, a decrease in ejection fraction, hypokinesia of the posterior wall of the left ventricle and / or interventricular septum, signs of relative insufficiency of the mitral and / or tricuspid valves, increased pressure in the pulmonary artery, separation of the sheets of the pericardium and / or the presence of free fluid in pericardial cavities. 3) X-ray of the chest organs: with damage to the heart - an increase in the size of the shadow of the heart, an increase in the pulmonary pattern and focal shadows. When the lungs are damaged, there may be an increase and deformation of the lung pattern, heavy seals and cellular enlightenments (a picture of a “honeycomb” lung). X-ray examination of the joints. In the first months of the disease, the main indicator is epiphyseal osteoporosis. Then erosion appears. 4) In difficult cases, for the diagnosis of articular syndrome, computed tomography and magnetic resonance imaging are used to accurately assess the development of pannus, articular effusion, cartilage loss and meniscal hypotrophy. Tomography of the joints - gamma scintigraphy with technetium-99 determines local minimal changes.
  • 43. The severity of radiographic changes on the Steinbroker scale: Stage I - osteoporosis, mainly epiphyseal. Stage II - osteoporosis and initial cartilage destruction, narrowing of the joint space. Stage III - pronounced destruction of cartilage and bone, bone erosion. Stage IV - symptoms of stage III and ankylosis.
  • 45.
  • 46. • therapy begins immediately after the diagnosis of JRA; • the aggressiveness of immunosuppressive therapy should correspond to the aggressiveness of the course of the disease.
  • 47. GOALS OF JRA THERAPY: • Suppression of the inflammatory and immunological activity of the process; • Relief of systemic manifestations and articular syndrome; • Preservation of the functional ability of the joints; • Prevention or slowdown of joint destruction, disability of patients; • Achieving remission; • Improving the quality of life of patients; • Minimize the side effects of therapy.
  • 48. NON-DRUG THERAPY 1.Mode During the period of exacerbation, the motor mode is limited. Complete immobilization is contraindicated, as it contributes to the development of contractures, osteoporosis, ankylosis, and physical exercises help to maintain the functional activity of the joints. Useful: cycling, swimming, walking. Undesirable: running, jumping, active games. Exclude exposure to the sun and psycho- emotional overload.
  • 49. 2. Therapeutic exercise is the most important component of JRA treatment. Daily exercises are needed to increase the range of motion in the joints, eliminate flexion contractures, and restore muscle mass. In case of damage to the hip joints, traction procedures are prescribed for the affected limb, walking on crutches. During the development of coxitis and aseptic necrosis of the femoral heads, the movement of the patient without crutches is contraindicated. Physiotherapy exercises should be carried out in accordance with the individual capabilities of the patient.
  • 50.
  • 51. 3. Orthopedic correction Static orthoses such as splints, splints, insoles are used - they should be worn or put on in their free time and must be removed during the day to stimulate the muscular system during exercise, classes, occupational therapy. In case of severe osteoporosis in the cervical spine, it is mandatory to wear a head holder (soft, hard), and in case of osteoporosis of the thoracic and lumbar spine, it is necessary to wear a corset or reclining system.
  • 52.
  • 53. MEDICAL THERAPY 1. Symptom Modifying Therapy (anti-inflammatory, symptomatic): 1.1. Non-steroidal anti-inflammatory drugs. 1.2. GCS: local therapy (intra-articular), pulse therapy. 2. Disease modifying therapy (pathogenetic, basic) 2.1. Cytotoxic drugs: methotrexate, cyclosporine A, leflunomide. 3. Biological agents: infliximab, rituximab. 1.1. Non-steroidal anti-inflammatory drugs (NSAIDs).
  • 54. RATIONAL APPROACH TO NSAID THERAPY: • Reception mode: start treatment with the lowest dose, with good tolerance after 2-3 days, the dose can be increased, take after meals, after taking NSAIDs, do not lie down for 15 minutes in order to prevent esophagitis. • Refusal of the simultaneous use of 2 or more NSAIDs. • Preventive treatment of NSAID gastropathy - acid- regulating drugs: proton pump inhibitors (omeprazole), H2-blockers (famotidine, ranitidine)
  • 55. International title Tradename Dose mg/kg/day Multiplicity of reception Application, with age Diclofenac Voltaren Diclofenac Naklofen Ortofen 2-3 2-3 5 years Naproxen Naproxen Apranax Naprios 15-20 2 5 years Ibuprofen Ibuprofen Ibufen Nurofen 35-40 2-4 3 months Piroxicam Pyroxifer Feldoral 0,3-0,6 1-2 12 years Selective COX-2 inhibitors Nimesulide Nise, Nimulid 3-5 2-3 up to 6 years with caution Meloxicam Movalis, Meloxicam 0,3-0,5 1 15 years NSAIDs used to treat arthritis in children:
  • 56. • 1.2. Glucocorticosteroids (GCS) • Prednisolone Dose: • High doses — 0.6-1.0 mg/kg/day • Average doses - 0.3-0.6 mg / day • Low doses — 0.3-0.1 mg/kg/day The initial dose is 0.2-0.5 mg/kg/day (15 mg/day) for no more than 1 month until remission is achieved, then the dose is reduced to a maintenance dose (0.1-0.15 mg/kg) followed by withdrawal.
  • 57. 1.2.1. PULSE THERAPY GCS Indications: • Severe systemic manifestations in JRA: - myopericarditis; - pneumonitis, pleuropneumonitis; - polyserositis, vasculitis • JRA complications: - cardiopulmonary insufficiency; - macrophage activation syndrome; • Ineffectiveness of previous therapy.
  • 58. Pulse therapy mode: Methylprednisolone 5-10 mg/kg/administration (no more than 500 mg) in 200 ml of saline for 35-40 minutes for 2-5 days in a row. In case of inefficiency: - increase the duration of the GC pulse therapy — repeat the course of GC pulse therapy
  • 59. 1.2.2. LOCAL THERAPY OF CORTICOSTEROIDS (INTRA-ARTICULAR) • Indications: • Mono-, oligoarthritis of moderate or high local activity; • Predominant defeat of 1 or 2 joints in polyarthritis; • With JRA at the beginning of basic therapy with high local activity of 1-2 joints; • If there are contraindications for basic therapy as a temporary palliative method; • Prevention of deformity as a component of a rehabilitation program.
  • 60. CONTRAINDICATIONS: • local or systemic infection • Pronounced bone destruction • Severe periarticular osteoporosis • Difficult access to the joint • Pathology of the blood coagulation system • Ineffectiveness of previous local GC therapy
  • 61. The amount of GCS for intra-articular administration Joint The amount of the drug Large knee, ankle, shoulder 1-2 ml Medium Elbow, wrist 0,5-1 ml Small interphalangeal, metacarpophalangeal, metatarsophalangeal 0,1-0,5 ml Enter no more than 1 time per month
  • 62. 2. CYTOTOXIC THERAPY 2.1. Methotrexate Rational principles of methotrexate therapy Appointment in the early stages of the disease. Application for a long period. Correction of therapy in case of ineffectiveness with adequate terms of treatment. Prevention of side effects of methotrexate - the appointment of folic acid - 5 mg / week. The standard dose of methotrexate is 10 mg/m2/week (0.3-0.5 mg/kg) The calculation of the body area is made according to nomographic tables or the Kosteff formula: S = 4m+7 , where m is the patient's body weight
  • 63. RULES FOR PRESCRIBING CYCLOSPORINE • Appointment in a specialized hospital. • Double control of serum creatinine concentration before prescribing the drug. • Cancel NSAIDs or reduce the daily dose in half. • Do not use with drugs that enhance the nephrotoxicity of cyclosporine: - macrolides - aminoglycosides. • Initial dose — 3.5 mg/kg/day.
  • 64. • • The daily dosage is divided into 2 doses of 1.5 mg/kg every 12 hours. • • The effect develops in 1-3 months and reaches a maximum within 6-12 months. • • Increasing the dose is carried out with the ineffectiveness of the initial dose after 2 months of treatment, not more than 1 time in 14 days, 25 mg. • • Do not exceed a daily dose of 5 mg/kg. • Monitoring treatment with cyclosporine • • Physical examination - once a month • • Clinical and biochemical blood tests - once every 2 weeks - control of serum concentrations: - creatinine, - urea, - uric acid, - aminotransferases (ALT, AST), - bilirubin, - potassium; • • Consultation with an ophthalmologist, examination with a slit lamp for all children with JRA — 1 time in 3 months. • • Measurement of blood pressure every day.
  • 65. • 2.3. Leflunomide (Arava) is indicated for severe JRA, torpid to classical immunosuppressants. • Mechanism of action of leflunomide • • Leflunomide - acts as an immunomodulator (without a cytotoxic effect), since it has much less effect on other cells (when other cells divide, the accumulated reserves of pyrimidine precursors are used). • Additional mechanisms: • - Inhibits the activation of NF-kB (a mediator of pro- inflammatory genes). • - Blocks the expression of cell adhesion molecules. • - Reduces the production of free radicals. • - Influence on COX.
  • 66. 3. BIOLOGICAL AGENTS 3.1. Infliximab (Remicade) is a monoclonal antibody against TNF-a. Dose: 3-6 mg/kg per regimen: 0, 2, 6 and then every 8 weeks. The use of leflunomide in pediatric practice: it is most effective in the early stages of JRA in patients with oligo- and polyarticular variants. It can be used in children over 10 years of age in a highly specialized rheumatology hospital with the permission of the ethical committee and the informed consent of the parents.
  • 67. 3.2. Rituximab (MabThera) is a monoclonal antibody to CD20 receptors on the surface of B-lymphocytes. Dose: 375 mg/km2 per administration schedule: 0, 1, 2.3 weeks. Use in pediatric practice: in severe systemic JRA variants refractory to standard therapy.
  • 68. COMBINED IMMUNOSUPPRESSIVE THERAPY JRA 3 main types of combination therapy: • Initiation of treatment with one drug and the appointment of several immunosuppressants after 8-12 weeks while maintaining disease activity. • Start of treatment with combination therapy with transfer to monotherapy (after 3-12 months) with suppression of the activity of the process. • Carrying out combination therapy during the entire period of the disease.
  • 69. COMBINATION THERAPY OPTIONS: 1. Combined pulse therapy with high doses of methotrexate and methylprednisolone · Methylprednisolone 15-20 mg/kg for 3 consecutive days. Methotrexate - EBEWE 30 mg/m2 in 2nd infusion followed by 15 mg/m2 IM weekly and repeated quarterly pulse therapy. 2. Combined therapy with methotrexate and cyclosporine. Methotrexate - 12-15 mg / m2 / week orally or initially pulse therapy at a dose of 50 mg / m2 / week for 8 weeks, switching to an intramuscular injection at a dose of 15 mg / m2 / week. Cyclosporine A - 3.5-5 mg / kg / day. 3. Combination therapy with leflunomide and methotrexate. Methotrexate - 10-12 mg / m2 / week for 8 weeks. Leflunomide: patients weighing <30 kg - 50 mg/day for 3 consecutive days, then 10 mg/day; patients weighing >30 kg — 100 mg/day for 3 consecutive days, then 20 mg/day.
  • 70. DISPENSARY OBSERVATION • Observation in an outpatient setting is carried out by a pediatric rheumatologist, a pediatric cardiorheumatologist or a pediatrician who has completed an advanced training cycle in pediatric rheumatology, taking into account the recommendations of a specialized rheumatology department. • Physical examination is carried out once a month. • When treating with immunosuppressants, clinical and biochemical blood tests are performed once every 2 weeks (protein and fractions, urea, creatinine, bilirubin. ALT, AST, alkaline phosphatase, calcium, potassium, sodium). • · Analysis of immunological parameters is carried out 1 time in 3 months (IgG, IgM, IgA, CRP, RF, ANF). • ECG once every 3 months.
  • 72. • • 40-50% of children have a favorable prognosis • • 1/3 of patients have a disease; continuously recurs; • • 20% of patients develop severe destructive arthritis; • • 20% develop amyloidosis in adulthood; • • 65% have severe functional impairment. FORECAST OF JRA SYSTEM VARIANTS:
  • 73. JRA COMPLICATIONS 1. Amyloidosis - due to the constant circulation of immune complexes in the bloodstream. Amyloid is deposited in the walls of blood vessels, in the kidneys, liver, myocardium, and intestines, which leads to disruption of their functions.
  • 74. 2. Macrophage activation syndrome (MAS). Trigger factors: bacterial, viral (CMVI, HSV, etc.) infection, drugs (NSAIDs, gold salts) In JA, the clinical picture of CAM may resemble sepsis or exacerbation of the underlying disease: persistent fever, hepatosplenomegaly, cytopenia.