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JUVENILE IDIOPATHIC ARTHRITIS
Presented by: Samira Alesaeidi, MD
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Definition An autoimmune, noninfective, inflammatory joint disease
Arthritis in one or more joints Begins before the 16th birthday Persisting for 6 weeks or more Has no other known cause
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EPIDEMIOLOGY Incidence: Age of onset: Female/male: 2/1 to 3/1
JIA criteria: 1/10,000 yr Age of onset: First peak: 1 to 3 years (girls > boys) Second peak: 8 to 10 years (boys > girls) Female/male: 2/1 to 3/1
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Signs and symptoms The first manifestation: Limping
Morning limp that improves with time Reduced reduced physical activity, and poor appetite or deformity, rather than pain or swelling Pain, Morning stiffness, Toddler may no longer stand in the crib in the morning or after naps School absences
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Extra-articular Eye disease: Photophobia
Associated with inflammation in the front of the eye UK guidelines 6 weeks of JIA being diagnosed or suspected Screening Slit lamp features of inflammation cells in the AC Acute anterior uveitis with hypopyon Intraocular pressure, visual acuity (VA)
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Extra-articular Leg length discrepancy, genu valgum
Growth disturbance: may have reduced overall rate of growth Paradoxically: involve large joints grow faster Leg length discrepancy, genu valgum Muscle atrophy: muscle weakness Osteoporosis
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CLASSIFICATION Juvenile Idiopathic Arthritis (ILAR) Oligoarticular JIA
Polyarthritis (RF negative) Polyarthritis (RF positive) Systemic onset JIA Psoriatic arthritis Enthesitis-related arthritis Undifferentiated arthritis
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Classification
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Pathogenesis Trigger Genetic Background Abnormal Immune Response
Disease
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Triggering Factors ENVIRONMENT High parental income
Being an only child Fetal exposure to smoking (JIA in girls) Infectious agent (virus, bacteria?)
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Genetic Background HLA Non-HLA HLA-B27: spondyloarthropathy
HLA-A: oligoarthritis DRB1: ANA-positive cases HLA-DRB1: eye disease Non-HLA Polymorphism: IL6, PTPN 22
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Ag APC T Cell B Cell Tissue Damage CD40L Il-1β, TNF-α Il-6 CD40
ANA Serum immunoglobulins TNF, IL-1, IL-6, IL17 Collagenase ( from Pannus ) Circulating immune complexes Complement activation> PMN Tissue Damage
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PATHOGENESIS Hallmark is the tumor-like expansion of inflamed synovial tissue (pannus) Histology Thickened synovium (highly vascular) Hyperplasia of synoviocytes in the lining layer Dense infiltrate of inflammatory cells ( T cells, macrophages, B cells and NKC)
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CLINICAL FEATURES OF SUBTYPES OF JUVENILE IDIOPATHIC ARTHRITIS
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OLIGOARTHRITIS The most common form (50-60%) F/M: 4/1
Age: before 6 years four or fewer joints If ANA+, patient need routine eye exam every 3 months
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OLIGOARTHRITIS Clinical Manifestations Joints:
knees and ankles, Small joints of the hand Temporomandibular joint Shoulders are rarely involved
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OLIGOARTHRITIS Laboratory Features A positive ANA test (50% to 70%)
Elevated ESR & CRP (mildly or moderately) Mild anemia A high ESR may predict progression to the extended subtype
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Polyarticular 20-40 % of JIA Five or more joints Girl>boy
Smaller joints are affected Symmetrical RF +: 20% of JIA, HLA-DRB1∗0401, 9-12 y, Uveitis: unusual RF - : ANA + in 40% of patients, Uveitis occurs in 5% to 20%
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POLYARTHRITIS, RHEUMATOID FACTOR POSITIVE
Clinical Manifestations Arthritis: Aggressive, symmetric PIP, MCP, wrists and large joints Felty's syndrome Rheumatoid nodules (10%) Uveitis: unusual
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Systemic 10-20 % of JIA Arthritis, fever, salmon pink rash Male=Female
Age: 2-4 y Involves both large and small joints Fever and rash come and go May have internal organ involvement Hepatosplenomegaly, lymphadenopathy, serositis, hepatitis Polymorphism in macrophage migration inhibitory factor RF – ANA –
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SYSTEMIC ARTHRITIS Laboratory Features Very high CRP and ESR
leukocytosis (neutrophilia) Thrombocytosis Anemia Liver enzymes, ferritin, and coagulation screen may be abnormal in severe cases Polyclonal hypergammaglobulinemia RF and complement levels are normal or high
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Treatment intra-articular corticosteroid
MTX suppress joint inflammation TNF alpha blockers, such as etanercept Celecoxib
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Oral steroids (short course )
Methylprednisolone (IV) Multiple joint injections
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SYSTEMIC ARTHRITIS Mild: NSAIDs
Treatment Mild: NSAIDs Indomethacin is helpful for fever and pericarditis Severe cases, non responders: steroids Prednisolon (0.5 mg/kg) Methylprednisolone (30 mg/kg bolus) DMARDS (methotrexate and cyclosporine) these are less effective than in polyarthritis
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PRINCIPLES OF TREATMENT
GROWTH DISTURBANCES Poor growth Pubertal delay Osteoporosis Bisphosphonates Calcium and vitamin D Physical therapy Stretches Muscle building Joint protection
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OLIGOARTHRITIS Topical medications
Uveitis : Topical medications Glucocorticoids and mydriatics Resistant to topical therapy or side effects Methotrexate and anti-TNF-α agents
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PSORIATIC ARTHRITIS Arthritis and psoriasis or
Arthritis and at least two Dactylitis Nail abnormalities (>/= 2 nail pits, onycholysis) Family history of psoriasis in a first-degree relative Oligoarticular Uveitis in 15% of children
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ENTHESITIS-RELATED ARTHRITIS
Arthritis and enthesitis or arthritis or enthesitis with >/= 2 of: (1) Sacroiliac joint tenderness, or inflammatory lumbosacral pain (2) Positive HLA-B27 (3) Onset of arthritis in a boy 6 years old or older (4) Acute anterior symptomatic uveitis (5) History of AS, ERA, sacroiliitis, IBD, Reiter's syndrome, or acute anterior uveitis in a first-degree relative
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ENTHESITIS-RELATED ARTHRITIS
Clinical Manifestations Enthesitis Patella Achilles' tendon Plantar fascia Arthritis Hips, knees, or ankles (symmetric or asymmetric) Spinal symptoms are rare (At onset) Acute anterior uveitis
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Soft tissue swelling (a) Joint space widening (b) Periostitis (c)
Radiographic changes Early changes Soft tissue swelling (a) Joint space widening (b) Periostitis (c)
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Sublaxation Deformity Ankylosis Osteonecrosis
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Abnormal increased uptake of TMJ (synovitis)
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Osteopenia Growth arrest lines
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Guidelines for screening eye exams in JIA
> 7 years </= 6 years subtype Every 6 months for 4 years then annually Every 3-4months for 4 Years then every 6 months for 3 years Any category except sJIA ANA+ Any category except Sjia ANA- Annual Systemic
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