Juvenile rheumatoid arthritis The Juvenile Rheumatoid Arthritis JRA

Juvenile rheumatoid arthritis

• The Juvenile Rheumatoid Arthritis (JRA) — a chronic inflammatory disease of joints of the unknown etiology, being characterized difficult autoimmune pathogensс, steadily progressing current, possible involvement in process of an internal and frequent development of disability in children.

Epidemiology • Prevalence universal. People of any age are surprised. Annual incidence - 0, 02%. • Prevalence YRA (till 16 years) - 0, 01 -0, 001% more often YRA develops at children of 1 -3 years.

Ethiology • It is insufficiently studied. • The known role belongs to a bacterial infection, and also to viruses and mycoplasmas • Contributing factors: • tonsillitis • cholecystitises • pyelonephritises • cystitis

Pathogenesis • • • • Primary anti-gene � hyper production of changed Ig � transformation of changed Ig in and perception their immune system as alien � production plasmatic cages of a synovial cover of antibodies (a rheumatoid factor) � AG +AT reaction � formation of immune complexes � aggregation of platelets, allocation of biologically active agents, complement activation, stimulation of migration of neutrophils in a joint cavity

• Phagocytes neutrophils of immune complexes and emission of proteolytic enzymes in a sinovial fluid • Activation by sinovial liquid of mediators of an inflammation • Damage of cellular structures of a cartilage of chain reaction • Immune complexes can circulate in blood extra articulate manifestations of RA.

• • Changes in immune system: The T-cellular: T-cellular in peripheral blood, T-cellular in sinovial liquid. • The V-cellular: • V-cellular in sinovial liquid, it is possible, at the expense of their transformation in mature plazmocytes.

Pathological anatomy • The Sinovial cover of joints bulked up, is hyperemic, further its microfleecy growth in the form of a raincoat. Fibroz and sclerosis of capsules of joints, formation of fibrous solderings. CCC: diffusion or focal myocarditis, перикардит fibrous and adhesive character, rheumatoid vasculitis. • Kidneys: interweft nephrite. • Liver: inflammation and sclerosis in portal paths; dystrophy and parenchyma necrosis.

RA classification • • • A. Clinical-anatomic: I. RA, articulate form 1) polyarthritis 3) monoarthritis II. RA with defeat of other bodies and systems (RES, heart, a liver, a kidney, vessels, serous covers, eyes, lungs, etc. ). III. Steel's syndrome (malignant form). IV. Allergoseptical syndrome V. RA, borderline 1) diffusion diseases of connecting fabric 2) rheumatism

• • • B. Clinical options: 1) rheumatoid factor "+" (seropozitivity) 2) rheumatoid factor (-) (seronegativity) 1) the fast-progressing 2) progressiv 3) low rogressiv G. Extent of activity: I. the low II. average III. the high

Clinical picture: • • Pain, swelling, morning constraint. YRA clinical forms: 1) articulate form 2) articulate visceral a) Steel's syndrome b) allergoseptical option c) option with separate 3) the form proceeding with defeat of eyes.

• For YRA defeat first of all large joints is characteristic, symmetry isn't peculiar. Mobility restriction in joints, a muscular atrophy, the general dystrophy, anemia accrues. Characteristic features YRA: delay of physical development, lag in growth, an underdevelopment of the bottom jaw.

Articulate visceral form • • • Includes five signs: Persistent high fever Polymorphic allergic rash Limfadenopatiya Gepatoliyenalny syndrome Arthritis This form has two main options: Still's syndrome Visslera-Fankoni's syndrome

Still's syndrome • articulate visceral form of YRA : fever with the daily peaks, a tremendous fever. Against fever: rash rozeolezy character. • The symptoms of arthritis first some weeks are absent. In an active phase - even the touch to joints is painful. Children are immobilized by sharply expressed pain syndrome and the general weakness. • At 10% - arthritises, are more often myocarditis. Tendency to progressing and frequent recurrence.

Diagnostic criteria of YRA • Clinical signs • 1. Arthritis, lasting 3 months and more; • 2. The arthritis of the second joint which has arisen in 3 months and later; • 3. Symmetric defeat of small joints; • 4. Contractures of joints; • 5. Muscular atrophy; • 6. Morning constraint; • 7. Rheumatoid defeat of eyes; • 8. Rheumatoid small knots; • 9 Exudates in a cavity of joints.

Differential diagnosis • Rheumatic arthritis • Arthritis • or artralgiya arise in 2 -3 weeks after transferred quinsy • Joints are surprised symmetrically • Pain and swelling keep from several days to 23 weeks • Arthritis passes without consequences

• Jet arthritis • Communication of arthritis with a certain infection • Asymmetric defeat of joints • Positive serological tests • Effect from antibacterial therapy

• • • Bekhterev's illness Slowly progressing mono - Often arthritis the coxofemoral joint is surprised Ekzostosis of calcaneal bones HLA B 27

• • • Reuters illness Communication with a hlamidy infection Asymmetric arthritis of the bottom arthritis extremities Reversibility of arthritis HLA B 27

Necessary researches: • 1) the general analysis of blood with calculation of platelets • 2) Sl. E • 3) S-jet protein • 4) general analysis of urine • 5) washout crops from a pharynx • In some cases: • 1) AIA and Ig definition • 2) definition of the Russian Federation, streptolyzin O and AT to an infecting agent Lima.

Laboratory changes: • The Russian Federation in blood serum is defined, isn't reliable as the false positive result can be received at hard currency, Shegren's syndrome, diseases of connecting fabric, at a virus and parasitic infection. • For YRA it is characteristic: hypokhromial anemia (Hb - 80 g / л 4 100 -110 g/l) because of the increased capture of Fe 2+РЭС. • High Sl. E, high credits of l indicators of an inflammation. Seldom at extra articulate manifestations Yl. RA cage LE-can find in patients.

Treatment • Landmark, complex. • Basis: the funds of pathogenetic influence allocated for suppression of inflammatory process and allergic reactions as a whole. And also local impact on articulate processes + sanitation of the centers of a chronic infection.

• 1) Acetilsalicylic acid: 75 -100 mg/kg/days, permanent improvement in 6 months. • 2) In the absence of reliable effect in 4 -6 months - basic means: preparation of gold, Dpenicillin, , pro-recension quinoline, cytostatics. • 3) SPP - glucocorticoids - only according to special indications and for short term as have many side effects.

• Indications to GKS application in treatment YRA: • 1) menacing lives pathological conditions (a carditis, myocarditis) • 2) persistent fever • 3) heavy forms of arthritis with the expressed restriction of mobility • 4) severe damages of separate joints (intra articulate introduction).

Prognosis • Rather favorable. • 1/3 patients as a result of a chronic progressing current have considerable restrictions of movements in joints, develops contractures. • Sharply worsens the forecast development secondary amyloidosis.

Prevention. • Primary specific prevention - No. • The nonspecific: sanitation of the centers of a chronic infection, medical supervision over children with the changed reactivity. For the recurrence prevention - dispensary supervision over children, suffering YRA is recommended.

JUVENILE RHEUMATOID THE ARTHRITIS • In the given lecture materials about features of current rheumatoid an arthritis at children are presented. Juvenile rheumatoid an arthritis - is destructive-inflammatory disease of joints from the unknown person etiology, complex pathogenesis, described a symmetric chronic arthritis, system defeat of the internal bodies, leading disabling the patients, developing at children in the age of till 16 years. • Among rheumatic illnesses of children's age JRA takes on prevalence the first place. Disease observe in various regions of globe with frequency from 0, 05 up to 0, 6 % in a population. • JRA - disease lifelong, however at correctly picked up therapy and regular supervision of the rheumatologist long remission with satisfactory quality of a life is possible. At frequent relapsing current, system displays of illness the forecast is more pessimistic - early occurs disability