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Rheumatic diseases in children. Yackov Berkun, Hadassah Mnt Scopus. Juvenile rheumatic diseases. A wide range of conditions Inflammatory , mechanical, behavioral or psychological May present with symptoms localized to the musculoskeletal system MS symptoms common in childhood - PowerPoint PPT Presentation
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Yackov Berkun, Hadassah Mnt Scopus
Rheumatic diseases in children
Juvenile rheumatic diseasesA wide range of conditionsInflammatory, mechanical, behavioral or
psychologicalMay present with symptoms localized to
the musculoskeletal systemMS symptoms common in childhood15% occasional limb pains5% chronic MS pain, daily activities
Lasting for 3 months or moreBerkun, JIA2
Juvenile rheumatic diseasesJuvenile idiopathic
ArthritisVasculitis
Kawasaki diseaseHenoch-Schonlein
purpuraAutoinflammatory
FMFPanniculitisCRMO/SAPHO
syndromesPFAPA
Connective tissueSLEJuvenile
dermatomyositisInflammatory bowel
diseaseMixed connective
tissue diseaseLinear sclerodermaProgressive
systemic sclerosis3
Mechanical/orthopedic Infection/post-infective
Viral rubella, measles, mumps, parvo, hepatitis
Reactive arthritis Rheumatic feverSeptic arthritisTransient hip synovitis
Hematological Idiopathic pain syndromesOtherBerkun, JIA4
Introduction, JIAMost frequent chronic inflammatory
disease of childhoodOne of the more common chronic
illnesses of childhood and an important cause of disability
First series 1890, George Stil 1897Group of disorders defined by ILAR
criteria 1997
Berkun, JIA5
EpidemiologyAll races and geographic areasPrevalence: 0.1-4/1000Chronic disease /1000
Epilepsy 3DM 1CP 1.3JIA 2
• 6 Berkun, JIA
ObjectivesTo recognize symptoms and signs of
clinical subgroups of JIATo understand laboratory and radiology
studies helpful when considering the diagnosis
To be familiar with the differential diagnosis
To understand the general approaches to treatment
7 Berkun, JIA
JRA American College of Rheumatology Revised Criteria
Age of onset < 16 yearsArthritis of one or more jointsDuration of disease > 6 weeks (EULAR 12)Other conditions which present with arthritis
in childhood must be excluded
8 Berkun, JIA
ILAR classification Criteria, Durban 1997
Oligoarthritis persistent extended
SystemicPolyarticular RF+Polyarticular RF-Psoriatic arthritisEnthesitis-related arthritis (ERA)Other
Berkun, JIA9
Oligoarthritis Most common 40-60% of JIA4 or fewer joints in the first 6 monthsInsidious onsetAge 1-3 yearsMonoarthritis 50%Large joints
Knee 50%, ankle, elbowAsymmetric Systemic symptoms absentUveitis 30%
10 Berkun, JIA
מפרקנפיחות
, בצקת, היפרטרופיה נוזל אודם ללא בתנועה הגבלה חד לא כאב בתינוק ביטוים
11 Berkun, JIA
12 • Berkun, JIA
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Oligoarthritis, Laboratory
CBC, ESR normalANA 60-80%
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30%ANAAsymptomatic50% at diagnosisWithin 5 yearsBilateral in 2/3Loss of vision 10%
Uveitis
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Uveitis- hypopion, synechiae
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Uveitis, Keratic precipitates
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Uveitis, compl. Band kerathopathy
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Uveitis, compl. Synechae
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Slit lamp exam
Oligoarthritis, outcome
• March 2010• Berkun, JIA23
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Oligoarthritis, outcome
ILAR classification Criteria, Durban 1997
Oligoarthritis persistent extended
Systemic onsetPolyarticular RF+Polyarticular RF-Psoriatic arthritisEnthesitis-related arthritis (ERA)Other
Berkun, JIA25
Systemic onset JIA10% of JIAFemale 40-50%Prominent systemic symptoms
fever, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, pleuritis
Arthritis may be absent for months to yearsUveitis uncommon
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Fever 2 weeks 390
QuotidianSpikes 1-2/d
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SoJIA, clinical features, fever
• Arthritis polyarticular• May be absent in 20%
Berkun, JIA 28
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Rash
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Rash
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Koebner
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SoJIA, clinical features, rashEvanescent nonfixed erythematousTrunk היקפי חיוורון חום עם מופיעהKoebner phenomena
34 Berkun, JIA March 2010
SoJIA, clinical features, general
SerositisPericarditis
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LymphadenopathyHepatosplenomegaly
Berkun, JIA36
• SoJIA, clinical features, general
Systemic JIA, criteria Arthritis + fever > 2 wks Quotidian > 3 days +1 of
evanescent nonfixed erythematous generalized lymphadenopathy hepato/ splenomegaly serositis
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SoJIA, laboratory
WBC , Hgb , platelets to ESR Ferritin ANA and RF negative
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ComplicationsHeart tamponadeMalnutritionOsteoporosisJoint deformityAmyloidosisGrowth retardation
Berkun, JIA39
Macrophage activation syndrome• Hemophagocytic lymphohistiocytosis• Life-threatening complication, cytokine
storm• NK cells dysfunction• Sustained fever, hepatosplenomegaly,
anemia, liver dysfunction, coagulopathy, CNS
• Early diagnosis• Aggressive treatment
Berkun, JIA • 40
Disease courses
Berkun, JIA41
Persistent polyarthritis (50%)Monocyclic, remission within 2–4 years
(40%) Relapsing polycyclic
Flares systemic + mild arthritis (10%)Poor outcomeSystemic features> 0.5 years,
thrombocytosis, polyarthritis incl. hip involvement
Mortality (2.8% to 14%)
ILAR classification Criteria, Durban 1997
Oligoarthritis persistent extended
SystemicPolyarticular RF-Polyarticular RF+Psoriatic arthritisEnthesitis-related arthritis (ERA)Other
Berkun, JIA42
Polyarticular, fingers
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Finger tenosynovitis
44 • Berkun, JIA
Berkun, JIA45
Polyarticular disease, laboratory
WBC , Hgb , platelets WNL to ESR to ANA 25%
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Polyarthritis RF+
5-10%Age >10 yearsSymmetricRheumatoid nodulesVasculitisLung
47 Berkun, JIA March 2010
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• Berkun, JIA
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Berkun, JIA50
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• Berkun, JIA
Micrognatia
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ILAR classification Criteria, Durban 1997
Oligoarthritis persistent extended
SystemicPolyarticular RF-Polyarticular RF+
Psoriatic arthritisEnthesitis-related arthritis (ERA)Other
Berkun, JIA53
Psoriatic arthritis
Arthritis and psoriasisDactylitis
sausagelike swelling of fingerNail pittingOnycholysisFamily historyAnterior uveitis, symptomatic
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PsoriasisTypical lesion -sharply
demarcated erythematous plaque covered by silvery white scales, on elbow
Initial eruptions- guttate distribution, often triggered by streptococcal infections
Scalp – 50%, beyond terminal hair
Nail pitting
• 56 • Berkun, JIA
Dactylitis
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Enthesitis related arthritisEnthesitis - inflammation at the insertion
of tendons, ligaments, or joint capsules to the bone
Arthritis lower limb, hip, intertarsalMale >8yLater SIJ, axial involvementHLA B27Familial historyAnterior uveitis, symptomatic
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XR , תהליך טראומה לשלול
אחר המרווח הרחבת עצם דלדול סחוסית תת בעצם כיב- הצרות סחוס איבודsacroiliitis
• 61
• Berkun, JIA
Tecnecium scan
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Treatment
• Nonsteroidal anti-inflammatory (NSAIDs)
Berkun, JIA 63
Treatment, Corticosteroids
Uncontrolled or life-threatening SoJIA
Local – uveitis, Intra-articularlar
Berkun, JIA
Treatment, MethotrexateMost widely used DMARD
Mostly in polyarticular disease
Oral, IM or SC
Adverse events/monitoring
stomatitis, leukopenia, nausea/ abdominal pain, anorexia,
malaise, fatigue, elevated hepatic enzymes, lung
Sulphasalazine
Berkun, JIA
Choy EH. N Engl J Med. 2001;344:907–916.
Inhibition of Cytokines
Activation ofanti-inflammatory pathways
Anti-inflammatory
cytokine
Suppression of
inflammatory
cytokines
Neutralization of cytokines
Soluble receptor
Monoclonal antibody
No signal
Receptor blockade
Monoclonal antibody
Receptor antagonist
No signal
Inflammatory cytokine
Normal interaction
Cytokine
receptor
Inflammatory signal
Biologicals
Berkun, JIA67
Anti TNFEtanercept – EnbrelInfliximab – RemicadeAdalimumab – Humira
Anti-IL-1; anakinraAnti-IL-6 (tocilizumab)T cell co-stimulation inhibitor
abatacept
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Orencia
Rilonacept (IL-1 receptor) Canakinumab. Anakinra- IL-1 receptor antagonist
Berkun, JIA70
Treatment, splint
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• Berkun, JIA
