Samatha Madhavarapu
HPI• Intermittent limp of R leg started 6 weeks ago.• Constant limp since 3 days• Worse upon awakening• Stiffness in R knee.• Transient warmth and redness +• Not able to bear weight initially, improves over
2 hrs.• Was outdoors in Upstate at BBQ 8 weeks ago• No fever, rash, recent URI• No trauma, diarrhea,
HPI Contd…PMH: NoneFH: grand father has seizure disorder. No
bone/joint problems Immunizations: UTDNKDAMeds: Tylenol PRN pain.Labs done 6 weeks ago: wbc7.6, 33.9/66.3,
ESR 18, xray R knee Normal.
Physical ExamVital signs:HEENT : NormalHeart , Lungs ,Abdomen: NormalSkin: No rashR knee: decreased extension, no swelling, no
redness, no warmth.Other Jts: FROM.
LABSBMP: 136/4.9/ 101/22/12/0.4/139/10.5Total Protein/ Albumin: 7.5/4.7LFT: 0.2/01, 38/19, 244CBC: 7.3/ 12/36.3/392ESR: 15
Other LabsCRP: 0.2ANA negativeLyme Ab titre: 1.2Anti CCP antibody: 8.3HLA B 27: NegativeRF: 20.0Ultrasound of Knees: Small R knee jt effusion
DD of Arthritis in ChildrenRheumatic &
Inflammatory Diseases
Seronegative Spondyloarthropathies
Infectious IllnessesReactive ArthritisImmunodeficiencies
Congenital & Metabolic Disorders
Bone & Cartilage Disorders
Neoplastic DisordersHematologic
DisordersPain Syndromes
Juvenile Rheumatoid ArthritisJRA most common rheumatic disease of
childhoodSynovitis of peripheral joints manifested as
swellingJRA is not a single disease, but a category of
diseases.
Criteria for Classification of JRA Age of onset: < 16 yrs Arthritis in > or = 1 joint Duration of the Disease: > or = 6 weeks Onset type is defined by type of articular
involvement in the first 6 months after onset:
Poly arthritis: > or = 5 inflamed joints Oligo arthritis: < or = 4 inflamed joints Systemic Disease: arthritis with intermittent
fever
Classification of Chronic Arthritis in Children
ACR, ELAR & ILAR classificationOnly ACR criteria have been
statistically validated.
Characteristic ACR ELAR ILAR
Onset Types 3 6 6
Age of onset of arthritis < 16 yr <16 yr <16 yr
Duration of arthritis >= 6 wk
>= 3mn
>= 6wk
Juvenile Ankylosing Spondylitis
Doesn’t include
includes
Includes
Juvenile Psoriatic Arthritis
Doesn’t include
includes
includes
Inflammatory Bowel Disease
Doesn’t include
includes
includes
Exclusion of other diseases
YES YES YES
Etiology• Unknown• Immunogenetic Susceptibility (Specific HLA
subtypes)• External Triggers - Viruses( EBV, Parvo virus B19, Rubella) - Hyperreactivity to self antigens(type 2
collagen) - Enhanced T-cell reactivity to heat schock
proteins of bacteria.
Epidemiology Incidence of JRA: 13.9/100,000 Sex: Pauci and poly articular disease more in girls Systemic onset –equal frequency in boys and girlsRace: Prevalnce of JIA lower in Urban African –
American compared to Caucasians Oligo 40% newly diagnosed cases in Caucasians. Blacks with JRA were older and less likely to test
positive for ANA or to have uveitis, more likely to test positive for Ig M RF
Pathogenesis• Synovitis: Villous hypertrophy & edema of
subsynovial tissues.• Vascular endothelial hyperplasia• Infiltration of mononuclear and plasma cells.• Pannus formation with erosion of cartilage and
bone.• Recriutment of T-cells specific to synovial non
specific antigens., facilitated by specific HLA types.
Clinical FeaturesOnset insidious or abruptMorning stiffness and gellingEasy fatiguabilityJoint pain (not very severe) and swelling,
limited joint movt, not erythematous.May have preceding illness
Oligoarthritis/Pauciarticular• Affects 4 or fewer joints• Typically larger joints (knees, ankles, wrists).• Starts with 1 joint• Monoarticular involvement of hip, upper
extremity large joints never presenting sign in JRA.
• If knee is affected-limping+, esp morning• Chronically- atrophy of extensor muscles of thigh,
tight hamstrings & knee flexion contractures.• Associated with HLA-DR8
Polyarthritis/Polyarticular• Minimum 5 joints should be effected.• Both large & small jts of upper and lower
extremities• Resembles adult RA and HLA profile.• Associated with HLA –DR4• Rheumatoid nodules in severe form• Micrognathia- chronic TM joint disease• C-Spine involvement- atlantoaxial
subluxation
Systemic Onset• Arthritis with visceral involvement• Characteristic intermittent spiking fevers to
>/= 39c for >/= 2 weeks. • Febrile episode assoc with evenescent (< 1
hr) macular rash, linear or circular, 2-5 mm, over trunk & proximal extremities.
• Arthralgia, myalgia• Hepatosplenomegaly,Lymaphadenopathy• Serositis/pericardial effusion• Photophobia (uveitis), irregular iris due to
synechiae
LabsCBC with diff:
Lymphopenia,Thrombocytosis,microcytic anemia. Neutropenia is uncommon.
ESR: - Always elevated with systemic JRA. - Usually elevated with polyarticular but
within reference range in pauci articular. - When elevated, ESR helps to monitor
success of medical treatment
Labs contdANA:Positive in 40-85% with oligo/poly articularUnusual with systemic onsetTitres do not correlate with disease
severity.Associated with incraesed risk of uveitisRF: Rare in systemic JRA. Marker of persistence of polyarticular JRA
into adulthood., devpt of rheumatoid nodules and poor functioning.
Total protein and albumin: levels are often decreased during active disease
ALT test: to exclude hepatitis (viral or autoimmune) prior to starting NSAIDS
U/A: to r/o infection (trigger of JRA or transient postinfectious arthritis) and nephritis (seen in pts with SLE)
ImagingX-ray: When 1 jt is affected , to r/o osteomyelitis or
septic arthritis. Soft tissue swelling, regional osteoporosis,
osteopenia, sub chondral bone erosions, narrowing of cartilage spaces, fusion of nueral arches.
MRI synovial inflammation, early minimal changes seen
Echo cardiography: Serositis
DEXA: osteopenia
X rays
ManagementMultidisciplinary Team for care of childrenMain goal: maximize daily functioning ,
minimize drug toxicity.Key predictor of long term outcome is early
diagnosis and referral to rheumatology team.Diet: Include 3 servings of calcium rich foodsActivity: more active, better the prognosis
NSAIDS• Used to treat all subtypes of JRA (40-60%
children show improvement).• Mean duration for anti-inflammatory effect in
JRA- 30 days• Most with pauci and few with poly respond to
NSAID alone . • Rofecoxib , celecoxib (selective Cox-2 inhibitors)
~ similar to naproxen effectiveness• Adverse : nausea, decresead appetite, abd pain.
Less gastritis Naproxen induced pseudoporphyria
Methotrexate• Safest, most efficacious, least toxic of of 2nd
line agents for JRA.• Used in 60% patients with poly JRA• Inhibits DHFR, purine synthesis. • Pts unresponsive to PO MTX benefit from SC
or IM administration. • Well tolerated and less toxicity in children.• Pts who respond well have improved growth,
functionality, radiographic improvement.
• For overwhelmingly inflammatory or systemic illness.• Bridge therapy for those who did not respond to conventional therapy• Ocular control of uveitis (drops or injections)• Intra articular use :initial therapy in pts with
only 1 or 2 joint involvement• Improvement in symptoms in 2-3 days,
which last for at least 6 mo in 60% and 1yr in 45%
Glucocorticoids
Anti TNF alpha • Etanercept,: Only one approved for children• Fusion protein with TNF receptor monomer to Fc portion of Ig G1.• Administered SC twice weekly used in active
polyarticular JRA who fail MXT therapy. • JRA assoc-chronic uveitis that is
inadequately responsive to steroid therapy.• Not to be used with h/o chronic infections• R/o TB before starting rx
SulphasalazineImproved joint inflammation & labs
compared to placebo.GI irritation and rash.Steven johnson in pts with active systemic
JRA.CI in porphyria and G6PD deficiency
Prognostic features• Child with oligo: esp. girls, onset< 6yrs age
–chronic uveitis risk• Polyarticular: RF, rheumatoid nodules• Systemic onset: number of joints involved,
duration of inflammation, severity of arthritis.
• Limb length discrepancy, contractures,• Disability continues into adulthood in 20%• Chronic pain syndromes.• Psychological complications.
http://www.youtube.com/watch?v=NqyB-cTxvs8
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