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27 Cards in this Set
- Front
- Back
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Joint pain in kids: ddx
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trauma
infections -streptococcus -parvovirus B-19 -gonococcus -tuberculosis -Lyme (must have traveled to endemic area) reactive -enteric organisms tumors -leukemia -bone tumors (benign & malignant) serum sickness-like reaction Non-rheumatologic Musculoskeletal -benign nocturnal pains of childhood (growing pains) -benign joint hypermobility -mechanical pain syndromes -overuse syndromes -specific joints: --knee = femoral patellar syndrome --hips = slipped capital femoral epiphysis, avascular necrosis Rheumatologic -juvenile idiopathic/rheumatoid arthritis -systemic lupus erythematosus -juvenile dermatomyositis -vasculitis --Henoch Schonlein purpura --Kawasaki disease --polyarteritis nodosa -chronic recurrent multifocal osteomyelitis -periodic fever syndromes Hereditary Autoinflammatory Disorders/ Periodic Fevers -cryopyrin-associated syndromes --neonatal onset multisystem autoinflammatory disease (NOMID), Muckle-Wells syndrome, familial cold autoinflammatory syndrome -Familial Mediterranean fever (FMF) -tumor necrosis factor receptor-associated periodic syndrome (TRAPS) -hyperimmunoglobulin D with periodic fever syndrome (HIDS) |
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Joint pain plus fever ddx
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Systemic juvenile arthritis
Infection Acute Rheumatic Fever Lupus Malignancy Periodic Fever Syndrome |
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Joint pain plus rash ddx
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Lupus
Juvenile Dermatomyositis Infections -Acute Rheumatic Fever -Rickettsial Disease Systemic Juvenile Arthritis Vasculitis Malignancy |
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JIA: age at onset, minimum duration of arthritis, onset subtypes, RF, include spondyloarthropathies
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<16
6 wks Oligo Poly (RF-) Poly (RF+) Systemic Psoriatic Enthisitis related Poly (RF+) Yes |
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JIA: epidemiology
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studies are difficult to interpret due to heterogeneity in classification
0.8% appears in sibling pairs incidence: 0.83-2.26/100,000/year prevalence: 113/100,000 in USA girls: boys 2:1 |
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JIA: etiology
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unknown
heterogenous phenotypes 2 observations: -autoimmune disease -complex genetic trait --many genetic predispositions are within the major histocompatibility complex (MHC) region on chromosome 6 -not due to trauma --trauma may call attention to an already weakened joint |
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JIA: lab testing
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Laboratories do NOT diagnose arthritis
-Anti-nuclear antibody (ANA) -Rheumatoid Factor -HLA-B27 -CBC differential -Acute phase reactants -Synovial Fluid Analysis Useful for supporting diagnosis, prognosis, and monitoring toxicity of therapy Laboratories do NOT diagnose arthritis |
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JIA: ANA
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multiple studies have demonstrated ANA positivity in up to 22 % of children with musculoskeletal complaints in whom no autoimmune disease or arthritis was found
-in the absence of objective findings of arthritis, ANAs are poor screening tests for juvenile arthritis important in identification of children most at risk for chronic uveitis -girls under age 6 with pauci/oligoarticular disease are at increased risk for uveitis |
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JIA: RF
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poor screening test in children
seldom found in children < 7 years old more common in children with later age of onset, subcutaneous nodules or articular erosions, or aggressive joint disease children with high titer RFs may represent a subgroup distinct from seronegative children as they are more likely to develop unremitting, disabling disease during adult years |
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JIA: HLA-B27
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positivity does not establish a diagnosis, but in children may support a diagnosis of spondyloarthropathy
studies suggest that 50 -70% of children with spondyloarthropathy are positive not all children with spondyloarthropathy will develop ankylosing spondylitis (AS) -among adults with AS, 11% have onset in childhood similar to adults, 90% of kids with AS are HLA-B27 positive |
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JIA: Synovial fluid
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in general, arthrocentesis is not needed to support the diagnosis of JIA
the main purpose is to rule out infection, NOT rule in JIA children with septic joints are exquisitely tender with range of motion, while children with JIA have mild to moderate discomfort |
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Oligoarthritis demographics, onset, clinical features
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most common form of JIA
peaks 2-4 years of age female predominance onset -insidious -limp or joint swelling joint involvement -knee most common -ankle -hands and feet <10% -rarely hips fever is absent, CBC is unremarkable, erthrocyte sedimentation rate (ESR) may be normal to ↑ extended oligoarthritis: extension to >4 joints -involvement of other joints in the first 6 months -ESR >20 -involvement of wrists or other joints of upper extremities -symmetrical joint involvement |
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Uveitis
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most serious complication because may lead to blindness
20-30% of patients -highest risk among girls with +ANA insidious onset, usually painless |
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oligoarticular treatment and prognosis
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Treatment
-NSAIDs -intra-articular corticosteroid injection -sulfasalazine, methotrexate, biologics Prognosis -improved considerably in the last 15 years -about ½ of children the disease goes into remission without further flares |
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RF - polyarthritis: presentation and demographics
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affects 5 or more joints during the first 6 months of disease
least defined and most heterogenous form of JIA about ¼ of children with JIA have polyarticular disease at onset, and the vast majority are RF – 2 age peaks - toddler to pre-school - preadolescent |
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RF - polyarthritis: labs and complications
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acute phase reactants are usually elevated
may have a mild normocytic anemia 20 – 40% are ANA+ -higher risk of uveitis Complications -growth retardation is proportional to degree and duration of inflammation - ↓ bone mineral density |
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RF - polyarthritis: treatment and prognosis
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Treatment
-NSAIDs -methotrexate +/- biologics Prognosis -variable -symmetrical arthritis and early hand involvement appears to predict future disability -in 1 study, the probability of remission at 10 years was 23% -overall, worse than oligoarticular and better than RF+ polyarticular |
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RF + polyarthritis: demographics, prognosis
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smallest category of JIA, about 5% of cases
most common in teenage girls poor prognosis -uveitis uncommon may be considered the youngest end of the distribution of rheumatoid arthritis, but in the adolescent who is still growing, differences between adults and pediatrics do not allow direct comparison |
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Systemic JIA: diagnosis, age
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clinical diagnosis, as there is no specific diagnostic test
all age groups -adults referred to as “Still disease” -George Frederic Still described it as a distinct entity in children in 1897 proportion of children with JIA having this types varies from 4 to 13% |
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Systemic JIA: clinical manifestations
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extra-articular manifestations make it unique
-fever -rash -lymphadenopathy -organomegaly -serositis |
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Systemic JIA: complications
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macrophage activation syndrome
-rare potentially life-threatening hemophagocytic process -characterized by fever, hepatosplenomegaly, bruising, and coagulopathy joint destruction osteoporosis growth retardation -inflammation -corticosteroids |
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Systemic JIA: treatment, prognosis
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Treatment
-NSAIDs -corticosteroids -methotrexate -biologics Prognosis -up to 1/3 develop significant disability -risk factors for a poor prognosis are high platelet count and persistent symptoms after 6 months of treatment |
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Psoriatic: demographics, presentation
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represent 2- 15% of all children with JIA
dactylitis, nail changes, family history of psoriasis in a 1st degree relative uveitis, seen in up to 20% of children with psoriatic arthritis is similar to the pattern seen in children with oligoarticular JIA after years of follow-up, 40% have persisting disease |
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Enthesitis related: definition
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arthritis with enthesitis
enthesitis with at least 2 of the following: -sacroiliac joint tenderness -+HLA-B27 -family history in at least one first degree relative of confirmed HLA-B27 disease -acute anterior uveitis -onset of arthritis after the age of 6 years |
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Enthesitis related: epidemiology
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boys>girls
usually presents in late childhood and adolescence accounts for 4-15% of children with JIA strong association with HLA-B27 |
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Enthesitis related: clinical manifestations
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arthritis predominantly in the lower extremities
enthesitis particularly around the knee and foot occasionally low grade fever, weight loss, and fatigue |
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Enthesitis related: complications and prognosis
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Complications
-spinal fusion in childhood is rare -aortic valve insufficiency -acute anterior uveitis (symptomatic) Prognosis -in North America the evolution to more severe disease appears to be less than in other countries -long-term outcome data are lacking |
