pediatrics diseases

1. JUVENILE
IDIOPATHIC
ARTHRITIS

2. • JUVENILE IDIOPATHIC ARTHRITIS (JIA) IS THE MOST
COMMON RHEUMATOLOGIC DISEASE AMONG
CHILDREN.
• JIA IS A CLINICALLY HETEROGENEOUS GROUP OF
DISEASES CHARACTERIZED BY ARTHRITIS THAT
BEGINS BEFORE THE AGE OF 16, INVOLVES ONE OR
MORE JOINTS, AND LASTS AT LEAST 6-8 WEEKS.
• ARTHRITIS IS DEFINED AS A SWELLING OR A
COMBINATION OF THE 2 FOLLOWING SIGNS: PAIN ON
MOTION OR PALPATION, FUNCTION LIMITATION,
INCREASE OF LOCAL TEMPERATURE.
• JIA OFTEN PERSISTS INTO ADULTHOOD AND CAN
RESULT IN SIGNIFICANT LONG-TERM MORBIDITY,
INCLUDING PHYSICAL DISABILITY.

3. CLASSIFICATION
Several terms for juvenile arthritis definition were used. In accordance with the
American (ACR) classification all pediatric arthritis got the name “juvenile
rheumatoid arthritis’. European antirheumatic liege in 1984 offered the term
‘juvenile chronic arthritis”. In 1997 international antirheumatic liege combined
these trends and offered the term JIA. The diagnostic parameters can be seen

4. ETIOLOGY• THE ETIOLOGY OF JIA IS UNKNOWN
• THE MOST ACCEPTABLE THEORY SUPPORTS THE INFLUENCE OF IMMUNOGENIC MECHANISMS
SECONDARY TO DIFFERENT GENETIC AND ENVIRONMENTAL FACTORS. INFECTIONS, STRESS AND
TRAUMA, ARE CONSIDERED TO BE THE MOST RESPONSIBLE TRIGGERING FACTORS.
• THE INCREASED FREQUENCY OF AUTOIMMUNE DISEASES AMONG JIA PATIENTS SUGGESTS THE
GENETIC BASIS OF THE DISEASE. HUMAN LEUKOCYTE ANTIGEN (HLA) B27 AND THE OTHER HLA
TISSUE TYPES (A 2,5,10, DR8, 15) ARE THE MOST COMMONLY MENTIONED GENETIC FACTORS.
• IT IS CONSIDERED THAT VARIOUS INFECTIONS ARE RESPONSIBLE FOR JIA ONSET. MOST COMMON
INFECTION TRIGGERING FACTORS ARE:
• ENTERIC INFECTIONS,
• PARVOVIRUS B19,
• RUBELLA,
• MUMPS,
• HEPATITIS B,
• EPSTEIN-BARR VIRUS,
• MYCOPLASMA AND
• CHLAMYDIA INFECTIONS.

5. HLA-ANTIGEN IN PATHOGENESIS OF AUTOIMMUNE
DISEASE
Molecular mimicry hypotheses
Гипотеза модификации HLA-
антигенов
Macrofages
HLA
Bacterial antigen
AB
AT
Macrofagesг
вирусы
HLA
JIA whole B27, DR5, DR8
JIA,systemic
variant
A10, B14, B16,
B27, DR8, DR9
JIA oligo- and
poly arthritis
А3, В15, В22,
DR5, DR8
Entesit-related
arthritis
B27, DR4, DR1
Reactive arthritis
A3, B22, B27,
DR8

6. PATHOGENESIS
• POTENTIAL TRIGGERS INDUCE MACROPHAGES AND T-LYMPHOCYTE ACTIVATION. AS A RESULT,
INFLAMMATORY CYTOKINES ARE SECRETE, WHICH LEADS TO JOINT DESTRUCTION.
MACROPHAGES, INDUCED BY SECRETED MEDIATORS, PRODUCE PRO-INFLAMMATORY
CYTOKINES [INTERLEUKIN (IL) 1, IL-6, TUMOUR NECROSIS FACTOR (TNF)-Α]. THEN, SECRETION
OF INFLAMMATION MARKERS [C-REACTIVE PROTEIN (CRP) AND SOME ENZYMES INCREASE AND

7. OLIGOARTICULAR JUVENILE IDIOPATHIC
ARTHRITIS• OLIGOARTICULAR VARIANT OF JIA IS DEFINED AS ARTHRITIS IN
4 OR LESS JOINTS DURING THE FIRST 6 MONTHS OF DISEASE.
OLIGOARTICULAR JIA IS THE MOST COMMON FORM OF JIA
(40%). IT TYPICALLY OCCURS BEFORE AGE OF 5, AND AFFECTS
GIRLS MORE OFTEN THAN BOYS (AT A RATIO 4 TO 1). THE KNEE
IS THE MOST COMMONLY AFFECTED JOINT FOLLOWED BY THE
ANKLES. WRISTS, HIP JOINT, CERVICAL SPINE AND SMALL
JOINTS OF THE HAND. JOINTS BECOME ENLARGE, WARM AND
PAINFUL. CHILDREN OFTEN EXPERIENCE MORNING STIFFNESS,
PAIN AND LIMITATION OF MOTION, BUT UP TO 25% MAY HAVE
PAINLESS ARTHRITIS.
• THERE ARE TWO SUBTYPES OF OLIGOARTHRITIS; PERSISTENT
OLIGOARTHRITIS AFFECTS A MAXIMUM OF FOUR JOINTS
THROUGHOUT THE DISEASE COURSE, AND EXTENDED
OLIGOARTHRITIS AFFECTS MORE THAN FOUR JOINTS AFTER
THE FIRST 6 MONTHS OF DISEASE.
• AN EXTENDED DISEASE, CERVICAL SPINE AND HIP

8. • THIS VARIANT OF ARTHRITIS IS OFTEN ACCOMPANIED BY DAMAGE TO VISION. ASYMPTOMATIC
IRIDOCYCLITIS (ANTERIOR UVEITIS THAT AFFECTS THE IRIS AND CILIARY BODY) IS COMMON IN
OLIGOARTHRITIS. IT IS SPREAD PARTICULARLY AMONG YOUNG GIRLS WHO ARE ANTINUCLEAR
ANTIBODY (ANA) POSITIVE. THEY MUST BE SCREENED BY OPHTHALMOLOGIST (DURING SLIT
LAMP EXAMINATION) FOR DISEASE PRESENTATION AND SERIALLY (2-4 A YEAR) THEREAFTER.
COMPLICATIONS OF UVEITIS INCLUDE VISUAL IMPAIRMENT, POSTERIOR SYNECHIAE (WHICH
DEFORM THE PUPIL), CATARACTS, BAND KERATOPATHY AND GLAUCOMA.
Ocular
manifestati
on in JIA

9. POLYARTICULAR JUVENILE IDIOPATHIC
ARTHRITIS
• POLYARTICULAR JIA, IS ARTHRITIS IN FIVE OR MORE JOINTS DURING THE
FIRST 6 MONTHS OF DISEASE. IT IS THE SECOND MOST FREQUENT
SUBTYPE OF JIA (35-40%). IT IS DIVIDED INTO TWO SUBTYPES:
RHEUMATOID FACTOR (RF)-NEGATIVE AND RF-POSITIVE. A
SEROPOSITIVE TYPE OF DISEASE AFFECTS ADOLESCENT GIRLS MORE
FREQUENTLY THAN BOYS. IS CHARACTERIZED BY SYMMETRIC
INVOLVEMENT OF THE WRISTS, METACARPOPHALANGEAL JOINTS, AND
PROXIMAL INTERPHALANGEAL JOINTS, JOINTS DEFORMATION, WEIGHT
LOSS. IT LOOKS LIKE AN EQUIVALENT OF RHEUMATOID ARTHRITIS IN
ADULTHOOD. IT ALSO STARTS AS KNEE ARTHRITIS (IN 80-90% OF
PATIENTS) BUT DURING THE FOLLOWS SEVERAL WEEKS ARTHRITIS
EXTENDS AND ANOTHER JOINTS INVOLVE.

10. • BOUTONNIERE DEFORMITIES (INTERPHALANGEAL JOINT
FLEXION AND DISTAL INTERPHALANGEAL JOINT
HYPEREXTENSION), AND SWAN-NECK DEFORMITIES
(PROXIMAL INTERPHALANGEAL JOINT HYPEREXTENSION
AND DISTAL INTERPHALANGEAL JOINT FLEXION) ARE
FREQUENTLY SEEN IN SEROPOSITIVE PATIENTS.
• RHEUMATOID NODULES AND MILD SYSTEMIC
SYMPTOMS, SUCH AS LOW-GRADE FEVERS,
LYMPHADENOPATHY, AND HEPATOMEGALY ARE
COMMON IN SEROPOSITIVE POLYARTHRITIS.
• A SERONEGATIVE TYPE OF JUA IS MORE BENIGN,
AFFECTS YOUNGER GIRLS. ITS DEVELOPMENT IS
SUBACUTE AND GRADUAL. IT INVOLVES
PREDOMINANTLY THE LARGE AND SMALL JOINTS OF
THE HANDS AND FEET, THE CERVICAL SPINE, AND
TEMPOROMANDIBULAR JOINT (TMJ). THESE PATIENTS
ARE LIKELY TO DEVELOP UVEITIS.

11. • MICROGNATHIA RESULTS FROM
CHRONIC TEMPOROMANDIBULAR
JOINT DISEASE.
• CERVICAL SPINE INVOLVEMENT,
MANIFESTED AS DECREASED NECK
EXTENSION, LEADS TO A RISK OF
ATLANTOAXIAL SUBLUXATION AND
NEUROLOGIC SEQUELAE.
• HIP (PELVIC-FEMORAL) JOINT DISEASE
MAY PRESENT WITH A DECREASED OR
PAINFUL OF MOTION ON THE
EXAMINATION.
Radiography of cervical spine in JIA show
fusion of the neural arch between joints C2
and C3, narrowing and erosion of the
remaining neural arch joints, obliteration of
the apophyseal space and loss of the
normal lordosis

12. SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
• SYSTEMIC JIA IS DEFINED BY ARTHRITIS; FEVER FOR AT LEAST 2 WEEKS WITH HIGH
RECORDED SPIKES FOR AT LEAST 3 DAYS; AND AT LEAST ONE OF THE FOLLOWING:
ERYTHEMATOUS RASH, LYMPHADENOPATHY, HEPATOSPLENOMEGALY AND SEROSITIS
(PERICARDITIS OR PLEURITIS). THE DISEASE AFFECTS BOYS AND GIRLS EQUALLY AND
CAN OCCUR AT ANY AGE BUT MOST COMMONLY IN EARLY CHILDHOOD.
There are 2 subtypes of systemic JIA:
with persistent active polyarthritis (Still
diseases)
with oligoarthritic and active systemic
features

13. • CHILDREN OFTEN APPEAR ILL DURING FEVERS AND APPEAR SUBNORMAL
WHEN AFEBRILE. ARTHRITIS IS GENERALLY SYMMETRIC AND
POLYARTICULAR BUT MAY BE ABSENT AT ONSET. THE RASH CONSISTS OF
DISCRETE, SALMON-PINK MACULES THAT ARE MORE PRONOUNCED
DURING FEVER AND MAY BE ASSOCIATED WITH THE KOEBNER
PHENOMENON (LINEAR STREAKS ON THE SKIN ELICITED BY SCRATCHING).
• COMPLICATIONS OF SYSTEMIC JIA INCLUDE MACROPHAGE-ACTIVATING
SYNDROME (MAS), SYSTEMIC AMYLOIDOSIS, INFECTIONS (CAUSED BY
IMMUNOSUPPRESSION, AND CHRONIC CORTICOSTEROIDS THERAPY) AND
JOINTS DISABILITY.

14. • APPROXIMATELY 5% TO 8% OF CHILDREN
WITH SYSTEMIC JIA DEVELOP MAS. THIS
LIFE-THREATENING COMPLICATION IS
CHARACTERIZED BY ACUTE ONSET OF
SUSTAINED FEVER,
HEPATOSPLENOMEGALY AND
LYMPHADENOPATHY.
• LABORATORY ABNORMALITIES INCLUDE
PANCYTOPENIA, ELEVATION OF FIBRIN
DEGRADATION PRODUCTS AND
TRANSAMINASES,
HYPERTRIGLYCERIDEMIA,
HYPERFERRITINEMIA, LOW ERYTHROCYTE
SEDIMENTATION RATE (SECONDARY TO
HYPOFIBRINOGENEMIA), AND ELEVATED
Main Clinical, Laboratory,
and Pathologic Features of
Macrophage Activation
Syndrome

15. OUTCOMES OF SYSTEMIC FORM ARE
AMYLOIDOSIS (ACCOMPANIED BY RENAL
INSUFFICIENCY) AND SEVERE JOINT
DESTRUCTION WITH FUNCTIONAL
INCOMPETENCE (FAILURE) AND
DISABILITY.
PATIENT N. DISEASE DURATION-10 YEARS. THERAPY: NSAIDS, CORTICOSTERO

16. PSORIATIC ARTHRITIS
• PSORIATIC ARTHRITIS IS DEFINED BY PRESENCE
OF ARTHRITIS AND PSORIASIS, OR IF THE RASH IS
ABSENT, ARTHRITIS AND AT LEAST TWO OF THE
FOLLOWING SIGNS: DACTYLITIS (INFLAMMATION
OF THE BOTH ALL JOINTS AND EXTRAARTICULAR
TISSUES IN ONE FINGER/TOY), NAIL PITTING OR
ONYCHOLYSIS, AND PSORIASIS IN A FIRST-
DEGREE RELATIVE.

17. • THE DISEASE AFFECTS GIRLS MORE OFTEN THAN BOYS AND HAS A
BIMODAL ONSET, WITH PEAKS IN THE PRESCHOOL YEARS AND
EARLY ADOLESCENCE.
• THE ARTHRITIS IS USUALLY AN ASYMMETRIC OLIGO- OR
POLYARTHRITIS AFFECTING LARGE AND SMALL JOINTS OF LOWER
EXTREMITIES PREDOMINANTLY AND MAY DEVELOP SEVERAL YEARS
BEFORE OR AFTER SKIN AND NAIL DISORDERS.
• PATIENTS MAY BE ANA POSITIVE AND ARE AT RISK FOR
DEVELOPING UVEITIS.
• EXACERBATIONS OF JOINT SYNDROME ARE ASSOCIATED WITH
THE DYNAMICS OF SKIN CHANGES.

18. ENTHESITIS-RELATED ARTHRITIS (ERA)
• OVERLAPS WITH SPONDYLOARTHROPATHIES, JUVENILE ANKYLOSING SPONDYLITIS (JAS), AND
INFLAMMATORY BOWEL DISEASE (IBD)–ASSOCIATED ARTHRITIS. ENTHESITIS IS INFLAMMATION
AROUND THE ENTHESIS, THE SITE OF INSERTION OF LIGAMENTS, TENDONS, JOINT CAPSULE, OR
FASCIA TO BONES.
• THIS DISEASE IS DEFINED BY ARTHRITIS AND ENTHESITIS OR EITHER ARTHRITIS OR ENTHESITIS
WITH AT LEAST TWO OF THE FOLLOWING:
 the presence or a history of
sacroiliac tenderness or
lumbosacral pain;
 HLA-B27 antigen positivity;
 onset of arthritis in a male
after the age of 6 years;
 acute anterior uveitis;
 a first-degree relative with
ankylosing spondylitis,
sacroiliitis with IBD,
reactive arthritis, or acute
anterior uveitis.

19. • ON PHYSICAL EXAMINATION, ENTHESITIS IS IDENTIFIED BY TENDERNESS WHERE THE TENDONS
INSERT INTO THE BONES. SOME COMMON ENTHESITIS SITES INCLUDE THE INFERIOR POLE OF THE
PATELLA, ACHILLES TENDON INSERTION, PLANTAR FASCIA INSERTION, AND SACROILIAC JOINTS.
• PROGRESSION TO JUVENILE ANKYLOSIC SPONDYLITIS IS MOST LIKELY AMONG CHILDREN WITH
GENETIC PREDISPOSITION, EPISODIC ARTHRITIS OF THE LOWER EXTREMITY LARGE JOINTS,
ENTHESITIS, AND TARSITIS WITHIN 1 YEAR OF ERA SYMPTOM ONSET.
• EXTRAARTICULAR MANIFESTATIONS OF ERA INCLUDE SYMPTOMATIC ANTERIOR UVEITIS (RED,
PAINFUL, PHOTOPHOBIC EYE), AORTIC INSUFFICIENCY, AORTITIS, MUSCLE WEAKNESS, AND LOW-
GRADE FEVER. ERA CAN ALSO BE THE INITIAL MANIFESTATION OF INFLAMMATORY BOWEL DISEASE.
THUS, IT IS IMPORTANT TO SCREEN FOR GASTROINTESTINAL SYMPTOMS, GROWTH FAILURE
(WEIGHT LOSS), ERYTHEMA NODOSUM, AND APHTHOUS STOMATITIS.

20. SUMMARY
FEATURES OF JUVENILE IDIOPATHIC ARTHRITIS
SUBGROUPS

22. • BOTH THE NUMBER OF AFFECTED JOINTS AND THE SITES OF
INVOLVEMENT AFFECT THE DIFFERENTIAL DIAGNOSIS.
• ONLY TWO DISEASES FREQUENTLY CAUSE PROMINENT INVOLVEMENT
OF THE DISTAL INTERPHALANGEAL (DIP) JOINT: OSTEOARTHRITIS AND
PSORIATIC ARTHRITIS.
• EXTRA-ARTICULAR MANIFESTATIONS SUCH AS FEVER (EG, GOUT,
ENDOCARDITIS), RASH (EG, SYSTEMIC LUPUS ERYTHEMATOSUS,
PSORIATIC ARTHRITIS, STILL DISEASE), NODULES (EG, GOUT), OR
NEUROPATHY (EG, POLYARTERITIS NODOSA, GRANULOMATOSIS WITH
POLYANGIITIS) NARROW THE DIFFERENTIAL DIAGNOSIS.
DIFFERENTIAL DIAGNOSIS

23. DIAGNOSIS & EVALUATION
• IN THE PATIENT WITH ARTHRITIS, THE TWO CLINICAL SINGS ARE VERY IMPORTANT FOR
DIAGNOSIS: AFFECTED JOINT TYPES AND LOCATION (PATTERN), THE PRESENCE OR ABSENCE OF
EXTRA-ARTICULAR MANIFESTATIONS.
• THE JOINT PATTERN IS DEFINED BY THE ANSWERS TO THREE QUESTIONS:
1) IS INFLAMMATION PRESENT?
2) HOW MANY JOINTS ARE INVOLVED?
3) WHAT JOINTS ARE AFFECTED?
JOINT INFLAMMATION MANIFESTS AS SWELLING OR 2 OF 3 SIGNS: WARMTH, FUNCTIONAL
Main pediatric criteria

24. JOINTS
FUNCTION
ASSESSMEN
T

27. • TO COUNT ACTIVE JOINTS CAN BE USED TABLES OR SCHEMATIC HOMUNCULUS

28. • A RECENT SYSTEM OF JIA ACTIVITY ASSESSMENT IS CALLED “JUVENILE ARTHRITIS DISEASE
ACTIVITY SCORE” (JADAS). JADAS IS A DISEASE ACTIVITY INDEX THAT IS COMPOSED BY SUMMING
UP FOUR INDIVIDUAL CRITERIA:
• PHYSICIAN’S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (PGA),
• PARENT’S/PATIENT’S ASSESSMENT OF CHILD’S WELL-BEING (PPGA),
• COUNT OF JOINTS WITH ACTIVE ARTHRITIS (ASSESSED IN 71, 27, OR 10 JOINTS, DEPENDING ON
THE VERSION),
• ESR OR CRP.
• THE CALCULATION FOR THE DAS AND ITS MODIFICATIONS CAN BE DONE ONLINE AT:
HTTP://WWW.DAS-SCORE.NL/WWW.DAS-SCORE.NL/DASCULATORS.HTML
• THIS SCORE IS REQUIRED TO ASSESS WORSENESS OR IMPROVEMENT OF THE PATIENTS STATE AND

30. DEFINITION OF
IMPROVEMENT BY
ACR
• THE AMERICAN COLLEGE OF RHEUMATOLOGY (ACR) PEDIATRIC 30 CRITERIA ARE A SET OF
CRITERIA THAT ARE USED AS A PRIMARY OUTCOME MEASURE FOR TRIALS OF BIOLOGIC
THERAPIES AND FOR SECOND LINE THERAPIES. IT HAS BEEN PROSPECTIVELY VALIDATED.
• IT IS DEFINED AS A MINIMUM OF 30% IMPROVEMENT IN AT LEAST 3 OF THE CORE SET CRITERIA
AND NO MORE THAN ONE COMPONENT WORSENING BY >30%.
• THE ACR PEDIATRIC 20, 50, 70 AND 90 ARE ALSO DEFINED AS 20%, 50%, 70%, AND 90%
IMPROVEMENT RESPECTIVELY IN A MINIMUM OF THREE CORE SET CRITERIA WITH WORSENING
OF ONE VARIABLE BY NO MORE THAN 30%.
• THE ABOVE CRITERIA ARE GOOD FOR CLINICAL TRIALS BUT CAN NOT BE USED IN EVERYDAY
CLINICAL PRACTICE. BECAUSE THEY ARE UNABLE TO GIVE AN INDIVIDUAL’S DISEASE STATUS

31. ACR Classification Criteria of Functional Status in
JIA
Class I:
Completely able to perform
usual activities of daily living
(self-care, vocational, and
avocational)*
Class II:
Able to perform usual self-
care and vocational activities,
but limited in avocational
activities
Class III:
Able to perform usual self-
care activities, but limited in
vocational and avocational
activities
Class IV:
Limited ability to perform
usual self-care, vocational,
and avocational activities
*Self-care activities include
dressing, feeding, bathing,
grooming, and toileting.
Avocational (recreational and/or
leisure) and vocational (work,
school, homemaking) activities.

32. LABORATORY FINDINGS
• HEMATOLOGIC ABNORMALITIES OFTEN REFLECT THE DEGREE OF SYSTEMIC OR ARTICULAR
INFLAMMATION, WITH ELEVATED WHITE BLOOD CELL AND PLATELET COUNTS AND ANEMIA.
INFLAMMATION MAY ALSO CAUSE ELEVATIONS IN ESR AND C-REACTIVE PROTEIN.
• ELEVATED ANA TITERS ARE PRESENT IN 40-85% OF CHILDREN WITH OLIGOARTICULAR OR
POLYARTICULAR JIA, BUT ARE RARE WITH SJIA. ANA SEROPOSITIVITY IS ASSOCIATED WITH
INCREASED RISK OF CHRONIC UVEITIS IN JIA.
• ANTI–CYCLIC CITRULLINATED PEPTIDE ANTIBODY, LIKE RF, IS A MARKER OF MORE
AGGRESSIVE DISEASE. BOTH ANA AND RF SEROPOSITIVITY CAN BE CAUSED BY TRANSIENT
EVENTS, SUCH AS VIRAL INFECTION.
• CHILDREN WITH SJIA USUALLY HAVE STRIKING ELEVATIONS IN INFLAMMATORY MARKERS
AND WHITE BLOOD CELL AND PLATELET COUNTS. HEMOGLOBIN LEVELS ARE LOW, WITH
INDICES CONSISTENT WITH ANEMIA OF CHRONIC DISEASE.
• THE ESR IS USUALLY HIGH, EXCEPT IN MAS. ALTHOUGH IMMUNOGLOBULIN LEVELS TEND TO
BE HIGH, ANA AND RF ARE UNCOMMON. FERRITIN VALUES ARE TYPICALLY ELEVATED AND
CAN BE MARKEDLY INCREASED IN MAS (>10,000 NG/ML).

33. SYNOVIAL FLUID ANALYSIS
• SYNOVIAL FLUID ANALYSIS AND CULTURE SHOULD BE PERFORMED IN CHILDREN WITH
ACUTE JOINT SWELLING THAT IS ACCOMPANIED BY FEVER OR FOR WHOM THE DIAGNOSIS
IS UNCERTAIN.
• IN JIA, THE SYNOVIAL FLUID IS USUALLY YELLOW AND CLOUDY AND HAS DECREASED
VISCOSITY. SYNOVIAL FLUID LEUKOCYTE COUNTS ARE ELEVATED (TYPICALLY BETWEEN
15,000 AND 20,000 CELLS/MM3 BUT CAN BE HIGHER) WITH NEUTROPHIL PREDOMINANCE.
• IN CASE OF ACUTE JOINT SWELLING THE EVACUATION OF SYNOVIAL FLUID DECREASES THE
PRESSURE INSIDE JOINT AND RELIEFS PAIN. IN ADDITION TO THIS THE ARTICULAR LAVAGE
CAN BE PERFORMED. OR CORTICOSTEROID INJECTION CAN BE MADE AS AN ADDITIONAL
PROCEDURE.

34. ARTHROSCOPY AND SYNOVIAL
BIOPSY• ARE INVASIVE DIAGNOSTIC PROCEDURES AND NEED TO BE PERFORMED
IN SEVERE CASES OR WHEN DIFFERENTIAL DIAGNOSTICS IS DIFFICULT.
• ALL IMMUNOLOGIC ABNORMALITIES IN JIA CAUSE INFLAMMATORY
SYNOVITIS, CHARACTERIZED PATHOLOGICALLY BY VILLOUS
HYPERTROPHY AND HYPERPLASIA WITH HYPEREMIA AND EDEMA OF THE
SYNOVIAL TISSUE.
• VASCULAR ENDOTHELIAL HYPERPLASIA IS PROMINENT AND IS
CHARACTERIZED BY INFILTRATION OF MONONUCLEAR AND PLASMA
CELLS WITH A PREDOMINANCE OF T LYMPHOCYTES.
• ADVANCED AND UNCONTROLLED DISEASE LEADS TO PANNUS
FORMATION AND PROGRESSIVE EROSION OF ARTICULAR CARTILAGE
AND CONTIGUOUS BONE.

35. IMAGING
• STANDARD RADIOGRAPHS ARE USEFUL TO EVALUATE
PERIARTICULAR OSTEOPENIA, EROSIONS, FRACTURES
OR OTHER BONY ABNORMALITIES.
• AMONG CHILDREN WITH JIA, RADIOGRAPHIC
FEATURES INCLUDE SOFT TISSUE SWELLING, WIDENING
OR NARROWING OF THE JOINT SPACE, OSTEOPOROSIS,
EROSIONS, SUBLUXATION, OR ANKYLOSIS. TYPICAL
RADIOGRAPHIC FEATURES APPEAR IN 6-8 WEEKS
AFTER THE ONSET OF DISEASE
• EROSION USUALLY APPEAR AFTER 2 YEARS OF ACTIVE
DISEASE.
• CHILDREN WITH JIA ARE AT RISK FOR ATLANTOAXIAL
INSTABILITY, PARTICULARLY IN THE PRESENCE OF
ACTIVE CERVICAL DISEASE.

36. • ULTRASONOGRAPHY,
COMPUTING TOMOGRAPHY
AND MAGNETIC RESONANCE
IMAGING (MRI) MAY BE
USEFUL IN THE EVALUATION
OF JIA.
• ULTRASONOGRAPHY IS A
NONINVASIVE, QUICK, AND
INEXPENSIVE METHOD TO
CONFIRM A JOINT
EFFUSION.
• BONE SCANS ARE HELPFUL
TO DETECT OSTEOMYELITIS,
MALIGNANCY.
• MRI IS THE MOST SENSITIVE
TECHNIQUE TO IDENTIFY
EARLY EROSIONS.

37. ACR
CLASSIFICATION
CRITERIA FOR
DETERMINING
PROGRESSION OF JIA
STEINBROCKER O.
*THESE CRITERIA DESCRIBE
EITHER SPONTANEOUS
REMISSION OR A STATE OF
DRUG-INDUCED DISEASE
SUPPRESSION.

38. CLINICAL MANAGEMENT
• THE OPTIMAL APPROACH TO THE MANAGEMENT OF A CHILD WITH JIA IS BASED ON A
MULTIDISCIPLINARY TEAM: A PEDIATRIC RHEUMATOLOGIST, OPHTHALMOLOGIST, ORTHOPEDIC
SURGEON, MEDICAL NURSE, PHYSICAL THERAPIST AND PSYCHOLOGIST. NON-PHARMACOLOGICAL
AND PHARMACOLOGICAL INTERVENTIONS MAY AID IN THE MANAGEMENT OF JIA PATIENTS.
• THE GOALS OF TREATMENT ARE TO ACHIEVE DISEASE REMISSION, PREVENT OR HALT JOINT
DAMAGE, AND PROMOTE NORMAL GROWTH AND DEVELOPMENT. ALL CHILDREN WITH JIA
NEED INDIVIDUALIZED TREATMENT PLANS, AND MANAGEMENT IS DONE ACCORDING TO
DISEASE SUBTYPE AND SEVERITY, PRESENCE OF POOR PROGNOSTIC INDICATORS, AND
RESPONSE TO MEDICATIONS. DISEASE MANAGEMENT ALSO REQUIRES MONITORING FOR
POTENTIAL MEDICATION TOXICITIES

40. • NONSTEROIDAL ANTI-INFLAMMATORY DRUGS (NSAIDS) ARE THE FIRST LINE IN THE
TREATMENT OF JIA. NAPROXEN, , IBUPROFEN, DICLOFENAC, INDOMETHACIN, AND OTHERS
HAVE BEEN USED SUCCESSFULLY.
• NONSTEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS), HAVE BOTH ANALGESIC AND
ANTIINFLAMMATORY PROPERTIES. HOWEVER, MOST CHILDREN WITH JIA REQUIRE
ADDITIONAL THERAPY.
• NSAIDS CAN BE COMBINED WITH OTHER ANTIRHEUMATIC DRUGS IN PER ORAL OR REMEDY
FORMS (GEL, OINTMENT).
Nonsteroidal anti-inflammatory
drugs

41. • INTRAARTICULAR CORTICOSTEROID INJECTIONS ARE OFTEN
USED TO CONTROL OLIGOARTHRITIS LOCALLY.
• THE USE OF SYSTEMIC CORTICOSTEROIDS SHOULD OFTEN BE
AVOIDED OR MINIMIZED. SYSTEMIC STEROIDS ARE
RECOMMENDED ONLY FOR MANAGEMENT OF SEVERE SYSTEMIC
ILLNESS, FOR TRANSIENT (“BRIDGE”) THERAPY DURING THE
WAIT FOR THERAPEUTIC RESPONSE TO A DMARD, AND FOR
CONTROL OF UVEITIS.
• STEROIDS GIVE RISKS OF SEVERE TOXICITIES, INCLUDING
CUSHING SYNDROME, GROWTH RETARDATION, AND
OSTEOPENIA, AND THEY DO NOT PREVENT JOINT
DESTRUCTION.
• STEROIDS CAN BE ADMINISTERED IN SYSTEMIC JIA AS PUSH-
THERAPY (A SHORT LARGE-DOSE THERAPY COURSE):
Corticosteroids

42. • SECOND-LINE MEDICATIONS, SUCH AS HYDROXYCHLOROQUINE AND SULFASALAZINE, HAVE
BEEN USED IN PATIENTS WHOSE ARTHRITIS IS NOT COMPLETELY CONTROLLED WITH NSAIDS
ALONE.
• METHOTREXATE, GIVEN EITHER ORALLY OR SUBCUTANEOUSLY, HAS BECOME THE DRUG OF
CHOICE FOR POLYARTICULAR, EXTENDED OLIGOARTHRITIS AND SYSTEMIC-ONSET JIA.
METHOTREXATE CAN CAUSE BONE MARROW SUPPRESSION AND HEPATOTOXICITY; REGULAR
MONITORING CAN MINIMIZE THESE RISKS. LEFLUNOMIDE HAS ALSO BEEN USED, WITH A
SIMILAR ADVERSE EFFECT,.
Synthetic DMARDs

43. • BIOLOGIC AGENTS ARE THE LAST THIRD LINE OF THERAPY AND ARE CONSIDERED AS
PATHOGENETIC THERAPY.
• BIOLOGIC AGENTS THAT INHIBIT TNF-Α AND BLOCK THE INFLAMMATORY CASCADE,
INCLUDING ETANERCEPT, INFLIXIMAB, AND ADALIMUMAB. THEY ARE EFFECTIVE IN THE
TREATMENT OF JIA.
• THE RISKS OF THESE AGENTS ARE GREATER, HOWEVER, AND INCLUDE SERIOUS INFECTION
AND, POSSIBLY, INCREASED RISK OF MALIGNANCY. ANAKINRA - AN INTERLEUKIN-1
RECEPTOR ANTAGONIST IS VERY BENEFICIAL IN THE TREATMENT OF SYSTEMIC-ONSET JIA.
Biologic
agents

44. • BIOLOGIC MEDICATIONS THAT INHIBIT PROINFLAMMATORY CYTOKINES, SUCH AS TNF-Α, IL-
1, AND IL-6, DEMONSTRATED EXCELLENT DISEASE CONTROL. TNF-Α ANTAGONISTS (E.G.,
ETANERCEPT, ADALIMUMAB) ARE USED TO TREAT CHILDREN WITH AN INADEQUATE RESPONSE
TO METHOTREXATE, WITH POOR PROGNOSTIC FACTORS, OR WITH SEVERE DISEASE ONSET.
EARLY AGGRESSIVE THERAPY WITH A COMBINATION OF METHOTREXATE AND A TNF-Α
ANTAGONIST MAY RESULT IN EARLIER ACHIEVEMENT OF CLINICALLY INACTIVE DISEASE.
• WHEN SYSTEMIC SYMPTOMS DOMINATE SYSTEMIC STEROIDS ARE STARTED FOLLOWED BY THE
INITIATION OF IL-1 OR IL-6 ANTAGONIST THERAPY, WHICH OFTEN INDUCES A DRAMATIC
AND RAPID RESPONSE.
• CANAKINUMAB, AN IL-1Β INHIBITOR, AND TOCILIZUMAB, AN IL-6 RECEPTOR ANTAGONIST,
ARE FDA-APPROVED TREATMENTS FOR SJIA IN CHILDREN OLDER THAN 2 YR.

45. TREATMENT MANAGEMENT OF DIFFERENT TYPES
OF JIA
• CHILDREN WITH OLIGOARTHRITIS OFTEN SHOW PARTIAL RESPONSE TO NONSTEROIDAL
ANTIINFLAMMATORY DRUGS (NSAIDS), WITH IMPROVEMENT IN INFLAMMATION AND PAIN.
THOSE WHO HAVE NO OR PARTIAL RESPONSE AFTER 4-6 WK OF TREATMENT WITH NSAIDS
OR WHO HAVE FUNCTIONAL LIMITATIONS, SUCH AS JOINT CONTRACTURE OR LEG-
LENGTH DISCREPANCY, BENEFIT FROM INJECTION OF INTRAARTICULAR
CORTICOSTEROIDS. TRIAMCINOLONE HEXACETONIDE IS A LONG-LASTING PREPARATION
THAT PROVIDES A PROLONGED RESPONSE. A MINORITY OF PATIENTS WITH
OLIGOARTHRITIS SHOW NO RESPONSE TO NSAIDS AND INJECTIONS, AND THEREFORE
REQUIRE TREATMENT WITH DISEASE-MODIFYING ANTIRHEUMATIC DRUGS (DMARDS),
METHOTREXATE/ SULFASALAZINE/ LEFLUNOMIDE, AND, IF NO RESPONSE, TNF INHIBITORS.
• POLYARTHRITIS OR SJIA REQUIRES THE USE OF SYNTHETIC DMARDS AND/OF BIOLOGIC
AGENTS. CORTICOSTEROIDS CAN BE ADMINISTERED AS INTRAARTICULAR INJECTIONS OR
PUSH-THERAPY. SYSTEMIC STEROIDS ARE RECOMMENDED ONLY FOR MANAGEMENT OF
SEVERE SYSTEMIC ILLNESS. THE DURATION OF CORTICOSTEROIDS USE MUST BE LIMITED
WITH SWITCHING FOR MORE SAFE AND EFFECTIVE METHODS.

47. UVEITIS
• MANAGEMENT OF JIA MUST INCLUDE PERIODIC SLIT-LAMP OPHTHALMOLOGIC
EXAMINATIONS TO MONITOR FOR ASYMPTOMATIC UVEITIS.
• OPTIMAL TREATMENT OF UVEITIS REQUIRES COLLABORATION BETWEEN THE
OPHTHALMOLOGIST AND RHEUMATOLOGIST.
• INITIAL MANAGEMENT OF UVEITIS MAY INCLUDE MYDRIATICS AND CORTICOSTEROIDS
USED TOPICALLY, SYSTEMICALLY, OR THROUGH PERIOCULAR INJECTION. DMARDS ALLOW
FOR A DECREASE IN EXPOSURE TO STEROIDS, AND METHOTREXATE AND ANTIBODIES TO
TNF-Α (ADALIMUMAB AND INFLIXIMAB) ARE EFFECTIVE IN TREATING SEVERE UVEITIS.

48. RECOMMENDATIONS FOR
MEDICATION SAFETY
MONITORING

49. SURGICAL MANAGEMENT
• ORTHOPAEDIC SURGERY HAS A LIMITED ROLE IN THE MANAGEMENT PLAN FOR
MOST CHILDREN WITH JIA. HOWEVER, IN CASES OF REFRACTORY DISEASE OR
PERSISTENT LEG LENGTH DISCREPANCY, AN EXPERIENCED ORTHOPEDIC
SURGEON CAN HELP TO DESIGN AN INDIVIDUALIZED SURGICAL INTERVENTION.
POSSIBLE INTERVENTIONS INCLUDE ARTHROSCOPIC SYNOVECTOMY, SOFT
TISSUE RELEASE, EPIPHYSIODESIS (GROWTH PLATE FUSION), TOTAL
ARTHROPLASTY (JOINT REPLACEMENT), AND ARTHRODESIS (JOINT FUSION).

50. ACR Classification Criteria for Determinining
Clinical Remission in JIA
5 or more of the following present at least two consecutive months:
a. Morning stiffness < 15 minutes
b. No fatigue
c. No joint pain
d. No joint tenderness or pain on motion
e. No soft tissue swelling in joints or tendon sheaths
f. ESR (Westergren methold) < 30 mm/hour for a female or 20
mm/hour for a male
Exclusions: Clinical manifestations of active vasculitis, pericarditis, pleuritis or myositis, and unexplained
recent weight loss or fever attributable to rheumatoid arthritis will prohibit a designation of complete clinical
remission.

51. CLINICAL CASE: BEFORE TREATMENT
• PATIENT K. ______ YEARS
• DIAGNOSE:
• DISEASE
DURATION________

53. AFTER
TREATMENT
• BASIC THERAPY:
• TREATMENT COURSE_____

55. СИСТЕМНЫЙ АРТРИТ
Пациент Р. 8 лет. Диагноз: ЮРА, системный
вариант (синдром Стилла).
Вес - 18,5 кг (<5 центиля). Рост 116 см (<5
центиля). ИМТ – 13,9 (5 центиль). Окружность
талии 60 см (85 центиль для 6 лет). ,
Лечение – курсы ПТ МП и МТ, МТ и ЦсА постоянно

60. RECOMMENDATIONS FOR INITIATION OF BIOLOGIC
AGENTS