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Arthritis, Juvenile Idiopathic, Emergency Medicine


Basics


Description


  • Previously called JRA
  • JIA comprises persistent, unexplained arthritis lasting >6 wk, occurring <17 yr of age, and affecting a heterogeneous group of children.
  • Prevalence up to 1 in 1,000 children
  • Girls > boys for most forms
  • Classified into 7 subgroups: Systemic onset, polyarticular RF+, polyarticular RF-, pauciarticular, psoriatic, enthesitis, other or unclassified.
  • Subtypes are based on number, type, and symmetry of joints involved; presence of systemic symptoms; skin involvement; family history; and lab values.
  • Up to 20% of JIA patients remain unclassified or are classified in multiple categories.
  • Natural course of the disease depends on the subtype (pauciarticular with overall best prognosis), but full resolution occurs in <50% of JIA patients.
  • Many will have a fluctuating course and ongoing disease through adulthood.

Etiology


Believed to be an autoimmune disease triggered by an unknown environmental trigger in a genetically susceptible host  

Diagnosis


Subtypes


  • Systemic onset:
    • 10% of cases, girls = boys
    • Associated fever and arthritis:
      • Fever: Diurnal (>39 °C) of >2 wk duration, child looks ill during temperature spike
      • Arthritis: May involve any number of joints and may appear only weeks to months after onset of fever
    • In addition there must be 1 of the following:
      • Maculopapular, salmon-colored rash on trunk and axillae
      • Lymphadenopathy
      • Hepatosplenomegaly
      • Serositis
    • Most acute, ill appearing of subtypes
    • Destructive arthritis >50%

  • Systemic onset JIA patients are at risk for macrophage activation syndrome (MAS).
  • MAS is a proliferation of macrophages causing a DIC-like picture with resultant fever, mucosal bleeding, neurologic changes and multisystem failure.
  • ESR may be normal in active MAS
  • MAS mortality is 8-22%.
  • Pauciarticular:
    • 50% of cases of IJA
    • 80% girls, peak incidence 2-4 yr olds
    • Insidious onset and child appears healthy
    • ≤4 joints involved at 6 mo:
      • Involves larger joints, (89% knee) hip rarely affected
      • Joints swollen, mildly tender, with decreased range of motion (ROM), possible leg-length discrepancy
    • Uveitis in about 20%; no other systemic signs
    • Subset termed extended pauciarticular progresses to greater joint involvement after 6 mo and has worse prognosis
  • Polyarticular:
    • 10-30% of cases, girls > boys, bimodal peaks: 2-5 and 10-14 yr
    • >4 joints involved at 6 mo:
      • Arthritis often symmetrical, small or large joints-commonly knees, wrists, and ankles
      • Decreased ROM of cervical and lumbar spine and temporomandibular joint (TMJ)
    • Systemic involvement rare except for fatigue and anemia
    • Older girls with RF+ often go on to develop typical adult rheumatoid arthritis (RA) and are placed in a separate subtype.
  • Psoriatic:
    • Arthritis; asymmetric large joints of lower extremities and back
    • Psoriatic rash in patient or 2 of the following: Dactylitis, nail pitting, psoriatic rash in 1st-degree relative
    • Enthesitis-related (enthesis means pain at the insertion of a muscle or tendon)
    • Arthritis; asymmetric large joints of lower extremities
    • Boys > girls, age usually >6 yr
    • Sacroiliac (SI) joint pain
    • Limited flexion of lumbar spine
    • Uveitis
    • Often FH
  • Otherwise unclassified:
    • Arthritis not fitting into any distinct category

History
  • Findings based on specific subtype
  • Classic presentation is insidious arthritis, worse in AM and with periods of immobility, improved with ROM.
  • New-onset systemic subtype most likely to use ED because they appear acutely ill, whereas other subtypes have a more insidious onset.

  • Child with severe pain and red-hot joint probably does not have new-onset JIA.
  • Rapid onset of polyarticular joint involvement is atypical for JIA; infectious or reactive cause of arthritis should be ruled out.
  • Beware of occult infection in patients on immunosuppressants.

Physical Exam
  • Determine if child is systemically ill: Search for fever, rash, or other nonarthritic involvement.
  • Do careful joint evaluation, documenting the number of joints involved and noting whether they are red, warm, and swollen or have limited ROM.

Essential Workup


  • Rule out septic joint and malignant bone tumor.
  • Rule out other identifiable causes of joint inflammation.
  • Rule out complications from long-term drug therapy.

Diagnosis Tests & Interpretation


Lab
  • CBC, ESR; if ill appearance, add blood cultures
  • Other labs if suspicious of specific subtype: rheumatoid factor (RF), antinuclear antibodies (ANA), HLA-B27, LFTs:
    • Systemic-ESR often elevated, leukocytosis, thrombocytosis, anemia, minor AST/ALT elevations, positive RF or ANA rarely seen, MAS may be associated with elevated LFTs and abnormal clotting factors but will often have normal ESR
    • Pauciarticular-common to have positive ANA in young girls; other labs usually normal; if anemic or elevated ESR, are probably misclassified or pauciarticular extended subtype
    • Polyarticular-may be anemic; if positive RF more likely to go on to adult RA, ESR may be elevated
    • Enthesitis-more likely positive HLA; presence of positive RF or positive ANA specifically excludes enthesitis subtype
    • Psoriatic arthritis-usually seronegative RF
  • Unfortunately, RF and ESR may also be elevated in acute infection unrelated to JIA.

Consider adding Lyme titer if new joint swelling in endemic area.  
Imaging
  • Joint radiograph:
    • Early presentation: Soft tissue swelling, joint effusion
    • Late presentation: Osteoporosis, joint destruction, early growth plate closure
  • Ultrasound:
    • Evaluate for small effusion, especially if tap considered.

Diagnostic Procedures/Surgery
Arthrocentesis if concern for septic arthritis: 5,000-8,000 WBC/mm3 with negative Gram stain and culture typical for JIA  

Differential Diagnosis


  • Trauma
  • Infection:
    • Septic arthritis, viral infection (especially parvovirus), Lyme disease, rheumatic fever, tuberculosis, subacute endocarditis, malaria, Neisseria gonorrhoeae infection
  • Other rheumatic/connective tissue diseases:
    • Systemic lupus erythematosus, polyarteritis nodosa, Henoch-Sch ¶nlein purpura, sarcoid
    • Legg-Calv ©-Perthes disease/slipped capital femoral epiphysis
  • Neoplasm:
    • Be suspicious of neoplasm in a severely uncomfortable child with midshaft bone pain.
  • Hematologic disease:
    • Sickle cell disease, hemophilia
  • Drug reactions

Treatment


Initial Stabilization/Therapy


Toxic-appearing children: IV access, O2  

Ed Treatment/Procedures


  • ED treatment is directed toward ruling out a septic joint and other causes of acute arthritis
  • If the diagnosis of JIA is already established and the child presents with an acute flare, a treatment plan/medication adjustment should be coordinated with the childs rheumatologist.

Medication


  • Medication in children with JIA is geared toward eliminating clinical signs of active disease, maximizing joint function, and preserving growth. (Chronic inflammation can make affected limb slightly longer.)
  • Efficacy depends on JIA subtype and disease severity.
  • Most pauciarticular JIA responds to NSAIDs and joint injections; polyarticular and systemic JIA usually require disease-modifying antirheumatic drug (DMARD) therapy and/or biological agents.
  • Antibiotics are indicated only if the joint is infected.

As early aggressive therapy may prevent some of the long-term complications of JIA, it is now common for children to be placed on DMARDS and biological agents early in the disease course. These medications have serious potential side effects, including:  
  • Immunosuppression
  • Decreased vaccine response (live vaccinations are contraindicated)
  • Increased potential for malignancy
  • NSAIDs:
    • Responsiveness differs within NSAID subtype
    • Used alone in mild JIA subtype or with other medications
    • Ibuprofen: 30-45 mg/kg divided TID-QID
    • Naproxen: 10-20 mg/kg divided BID; maximum daily dose 1,000 mg
    • Side effects: Gastritis, hepatitis, renal, headache, dermatitis
    • Intra-articular steroids: Triamcinolone hexacetonide: 1 mL/mg of 20 mg/mL solution
    • Often provide long-term (6-18 mo) relief
  • DMARDs:
    • Include corticosteroids, methotrexate, sulfasalazine
    • Corticosteroids
    • Use judiciously because of long-term complications, but high-dose pulse therapy may be needed in acute attack.
    • Prednisone: 0.5-2 mg/kg PO
    • Methylprednisolone: 30 mg/kg daily IV up to 1 g for 1-5 days for high-dose pulse steroids
    • Side effects: Gastritis, adrenal suppression, osteopenia, Cushing syndrome, infection
    • Methotrexate: 5-15 mg/m2 PO/SC or IM per week
    • Considered 1st-line DMARD, as most will respond
    • Side effects: GI, nausea, liver toxicity, teratogenic
    • Sulfasalazine: 30 mg/kg/d divided QID
    • Poorly tolerated in up to 30%
    • Side effects: GI, rash, anorexia
    • Biological agents-engineered to target specific key cytokines, very expensive
    • Tissue necrosis factor binders
  • Etanercept: 0.8 mg/kg SC once a week
    • Adalimumab: < 30 kg: 20 mg, > 30 kg: 40 mg given SC administered every other week
    • Side effects: Infection, injection-site reactions, inhibit T-cell activation
    • Abatacept: 10 mg/kg infusion q4wk
    • Side effects: Infusion reaction, HA, cough, nausea, infection
  • Non-FDA-approved therapies:
  • Remicade, rituximab, anakira, leflunomide IL-1 and IL-6 blockers
  • Stem cell transplants are used rarely for severe cases unresponsive to medical treatment:
    • Treatment for MAS is nonstandardized but may include high-dose steroids, cyclosporine, cyclophosphamide, or intravenous immunoglobulin

Follow-Up


Disposition


Admission Criteria
Unclear diagnosis in ill-appearing child or if concern of secondary joint infection  
Discharge Criteria
  • No evidence of septic joint, systemic infection, or organ failure from drug therapy
  • Patient appears comfortable.
  • Appropriate follow-up has been arranged.

Issues for Referral
  • Orthopedics if septic joint suspected
  • Rheumatologist if meds need adjustment

Follow-Up Recommendations


  • Children need long-term consults with a rheumatologist.
  • Children with JIA need frequent eye exams to rule out uveitis (which is often asymptomatic until permanent damage has occurred).

Pearls and Pitfalls


  • Rule out acute joint infection (always consider Lyme disease in the appropriate geographic context).
  • Consider systemic onset JIA in child with prolonged diurnal febrile illness that is unresponsive to antibiotics.
  • Consider MAS in systemic onset JIA patients who appear septic.
  • Review patients medications to identify potential side effects or immunosuppression.

Additional Reading


  • Beresford  MW, Baildam  EM. New advances in the management of juvenile idiopathic arthritis-1: Non-biological therapy. Arch Dis Child Educ Pract Ed.  2009;94:144-150.
  • Beresford  MW, Baildam  EM. New advances in the management of juvenile idiopathic arthritis-2: The era of biologicals. Arch Dis Child Educ Pract Ed.  2009;94:151-156.
  • Espinosa  M, Gottlieb  BS. Juvenile idiopathic arthritis. Pediatr Rev.  2012;33:303-313.
  • Gowdie  PJ, Tse  SM. Juvenile idiopathic arthritis. Pediatr Clin N Am.  2012;59:301-327.
  • Marzan  KA, Shaham  B. Early juvenile idiopathic arthritis. Rheum Dis Clin Am.  2012;38:355-372.

See Also (Topic, Algorithm, Electronic Media Element)


  • Septic Joint
  • Lyme Disease

Codes


ICD9


  • 714.30 Polyarticular juvenile rheumatoid arthritis, chronic or unspecified
  • 714.31 Polyarticular juvenile rheumatoid arthritis, acute
  • 714.32 Pauciarticular juvenile rheumatoid arthritis
  • 714.33 Monoarticular juvenile rheumatoid arthritis
  • 714.3 Juvenile chronic polyarthritis

ICD10


  • M08.00 Unsp juvenile rheumatoid arthritis of unspecified site
  • M08.3 Juvenile rheumatoid polyarthritis (seronegative)
  • M08.90 Juvenile arthritis, unspecified, unspecified site
  • M08.20 Juvenile rheumatoid arthritis with systemic onset, unsp site
  • M08.011 Unspecified juvenile rheumatoid arthritis, right shoulder
  • M08.012 Unspecified juvenile rheumatoid arthritis, left shoulder
  • M08.019 Unsp juvenile rheumatoid arthritis, unspecified shoulder
  • M08.01 Unspecified juvenile rheumatoid arthritis, shoulder
  • M08.021 Unspecified juvenile rheumatoid arthritis, right elbow
  • M08.022 Unspecified juvenile rheumatoid arthritis, left elbow
  • M08.029 Unspecified juvenile rheumatoid arthritis, unspecified elbow
  • M08.02 Unspecified juvenile rheumatoid arthritis of elbow
  • M08.031 Unspecified juvenile rheumatoid arthritis, right wrist
  • M08.032 Unspecified juvenile rheumatoid arthritis, left wrist
  • M08.039 Unspecified juvenile rheumatoid arthritis, unspecified wrist
  • M08.03 Unspecified juvenile rheumatoid arthritis, wrist
  • M08.041 Unspecified juvenile rheumatoid arthritis, right hand
  • M08.042 Unspecified juvenile rheumatoid arthritis, left hand
  • M08.049 Unspecified juvenile rheumatoid arthritis, unspecified hand
  • M08.04 Unspecified juvenile rheumatoid arthritis, hand
  • M08.051 Unspecified juvenile rheumatoid arthritis, right hip
  • M08.052 Unspecified juvenile rheumatoid arthritis, left hip
  • M08.059 Unspecified juvenile rheumatoid arthritis, unspecified hip
  • M08.05 Unspecified juvenile rheumatoid arthritis, hip
  • M08.061 Unspecified juvenile rheumatoid arthritis, right knee
  • M08.062 Unspecified juvenile rheumatoid arthritis, left knee
  • M08.069 Unspecified juvenile rheumatoid arthritis, unspecified knee
  • M08.06 Unspecified juvenile rheumatoid arthritis, knee
  • M08.071 Unsp juvenile rheumatoid arthritis, right ankle and foot
  • M08.072 Unsp juvenile rheumatoid arthritis, left ankle and foot
  • M08.079 Unsp juvenile rheumatoid arthritis, unsp ankle and foot
  • M08.07 Unspecified juvenile rheumatoid arthritis, ankle and foot
  • M08.08 Unspecified juvenile rheumatoid arthritis, vertebrae
  • M08.09 Unspecified juvenile rheumatoid arthritis, multiple sites
  • M08.0 Unspecified juvenile rheumatoid arthritis
  • M08.1 Juvenile ankylosing spondylitis
  • M08.211 Juvenile rheumatoid arthritis w systemic onset, r shoulder
  • M08.212 Juvenile rheumatoid arthritis w systemic onset, l shoulder
  • M08.219 Juvenile rheu arthritis w systemic onset, unsp shoulder
  • M08.21 Juvenile rheumatoid arthritis with systemic onset, shoulder
  • M08.221 Juvenile rheumatoid arthritis w systemic onset, right elbow
  • M08.222 Juvenile rheumatoid arthritis w systemic onset, left elbow
  • M08.22 Juvenile rheumatoid arthritis with systemic onset, elbow
  • M08.231 Juvenile rheumatoid arthritis w systemic onset, right wrist
  • M08.232 Juvenile rheumatoid arthritis w systemic onset, left wrist
  • M08.239 Juvenile rheumatoid arthritis w systemic onset, unsp wrist
  • M08.23 Juvenile rheumatoid arthritis with systemic onset, wrist
  • M08.241 Juvenile rheumatoid arthritis w systemic onset, right hand
  • M08.242 Juvenile rheumatoid arthritis with systemic onset, left hand
  • M08.249 Juvenile rheumatoid arthritis with systemic onset, unsp hand
  • M08.24 Juvenile rheumatoid arthritis with systemic onset, hand
  • M08.251 Juvenile rheumatoid arthritis with systemic onset, right hip
  • M08.252 Juvenile rheumatoid arthritis with systemic onset, left hip
  • M08.25 Juvenile rheumatoid arthritis with systemic onset, hip
  • M08.261 Juvenile rheumatoid arthritis w systemic onset, right knee
  • M08.262 Juvenile rheumatoid arthritis with systemic onset, left knee
  • M08.269 Juvenile rheumatoid arthritis with systemic onset, unsp knee
  • M08.26 Juvenile rheumatoid arthritis with systemic onset, knee
  • M08.271 Juvenile rheumatoid arthritis w systemic onset, right ank/ft
  • M08.272 Juvenile rheumatoid arthritis w systemic onset, left ank/ft
  • M08.279 Juvenile rheumatoid arthritis w systemic onset, unsp ank/ft
  • M08.27 Juvenile rheumatoid arthritis w systemic onset, ank/ft
  • M08.28 Juvenile rheumatoid arthritis with systemic onset, vertebrae
  • M08.29 Juvenile rheu arthritis w systemic onset, multiple sites
  • M08.2 Juvenile rheumatoid arthritis with systemic onset
  • M08.40 Pauciarticular juvenile rheumatoid arthritis, unsp site
  • M08.411 Pauciarticular juvenile rheumatoid arthritis, right shoulder
  • M08.412 Pauciarticular juvenile rheumatoid arthritis, left shoulder
  • M08.419 Pauciarticular juvenile rheumatoid arthritis, unsp shoulder
  • M08.41 Pauciarticular juvenile rheumatoid arthritis, shoulder
  • M08.421 Pauciarticular juvenile rheumatoid arthritis, right elbow
  • M08.422 Pauciarticular juvenile rheumatoid arthritis, left elbow
  • M08.429 Pauciarticular juvenile rheumatoid arthritis, unsp elbow
  • M08.42 Pauciarticular juvenile rheumatoid arthritis, elbow
  • M08.431 Pauciarticular juvenile rheumatoid arthritis, right wrist
  • M08.432 Pauciarticular juvenile rheumatoid arthritis, left wrist
  • M08.439 Pauciarticular juvenile rheumatoid arthritis, unsp wrist
  • M08.43 Pauciarticular juvenile rheumatoid arthritis, wrist
  • M08.441 Pauciarticular juvenile rheumatoid arthritis, right hand
  • M08.442 Pauciarticular juvenile rheumatoid arthritis, left hand
  • M08.449 Pauciarticular juvenile rheumatoid arthritis, unsp hand
  • M08.44 Pauciarticular juvenile rheumatoid arthritis, hand
  • M08.451 Pauciarticular juvenile rheumatoid arthritis, right hip
  • M08.452 Pauciarticular juvenile rheumatoid arthritis, left hip
  • M08.459 Pauciarticular juvenile rheumatoid arthritis, unsp hip
  • M08.45 Pauciarticular juvenile rheumatoid arthritis, hip
  • M08.461 Pauciarticular juvenile rheumatoid arthritis, right knee
  • M08.462 Pauciarticular juvenile rheumatoid arthritis, left knee
  • M08.469 Pauciarticular juvenile rheumatoid arthritis, unsp knee
  • M08.46 Pauciarticular juvenile rheumatoid arthritis, knee
  • M08.471 Pauciarticular juvenile rheumatoid arthritis, right ank/ft
  • M08.472 Pauciarticular juvenile rheumatoid arthritis, left ank/ft
  • M08.479 Pauciarticular juvenile rheumatoid arthritis, unsp ank/ft
  • M08.47 Pauciarticular juvenile rheumatoid arthritis, ankle and foot
  • M08.48 Pauciarticular juvenile rheumatoid arthritis, vertebrae
  • M08.4 Pauciarticular juvenile rheumatoid arthritis
  • M08.80 Other juvenile arthritis, unspecified site
  • M08.811 Other juvenile arthritis, right shoulder
  • M08.812 Other juvenile arthritis, left shoulder
  • M08.819 Other juvenile arthritis, unspecified shoulder
  • M08.81 Other juvenile arthritis, shoulder
  • M08.821 Other juvenile arthritis, right elbow
  • M08.822 Other juvenile arthritis, left elbow
  • M08.829 Other juvenile arthritis, unspecified elbow
  • M08.82 Other juvenile arthritis, elbow
  • M08.831 Other juvenile arthritis, right wrist
  • M08.832 Other juvenile arthritis, left wrist
  • M08.839 Other juvenile arthritis, unspecified wrist
  • M08.83 Other juvenile arthritis, wrist
  • M08.841 Other juvenile arthritis, right hand
  • M08.842 Other juvenile arthritis, left hand
  • M08.849 Other juvenile arthritis, unspecified hand
  • M08.84 Other juvenile arthritis, hand
  • M08.851 Other juvenile arthritis, right hip
  • M08.852 Other juvenile arthritis, left hip
  • M08.859 Other juvenile arthritis, unspecified hip
  • M08.85 Other juvenile arthritis, hip
  • M08.861 Other juvenile arthritis, right knee
  • M08.862 Other juvenile arthritis, left knee
  • M08.869 Other juvenile arthritis, unspecified knee
  • M08.86 Other juvenile arthritis, knee
  • M08.871 Other juvenile arthritis, right ankle and foot
  • M08.872 Other juvenile arthritis, left ankle and foot
  • M08.87 Other juvenile arthritis, ankle and foot
  • M08.88 Other juvenile arthritis, vertebrae
  • M08.89 Other juvenile arthritis, multiple sites
  • M08.8 Other juvenile arthritis
  • M08.911 Juvenile arthritis, unspecified, right shoulder
  • M08.912 Juvenile arthritis, unspecified, left shoulder
  • M08.919 Juvenile arthritis, unspecified, unspecified shoulder
  • M08.91 Juvenile arthritis, unspecified, shoulder
  • M08.921 Juvenile arthritis, unspecified, right elbow
  • M08.922 Juvenile arthritis, unspecified, left elbow
  • M08.929 Juvenile arthritis, unspecified, unspecified elbow
  • M08.92 Juvenile arthritis, unspecified, elbow
  • M08.931 Juvenile arthritis, unspecified, right wrist
  • M08.932 Juvenile arthritis, unspecified, left wrist
  • M08.939 Juvenile arthritis, unspecified, unspecified wrist
  • M08.93 Juvenile arthritis, unspecified, wrist
  • M08.941 Juvenile arthritis, unspecified, right hand
  • M08.942 Juvenile arthritis, unspecified, left hand
  • M08.949 Juvenile arthritis, unspecified, unspecified hand
  • M08.94 Juvenile arthritis, unspecified, hand
  • M08.951 Juvenile arthritis, unspecified, right hip
  • M08.952 Juvenile arthritis, unspecified, left hip
  • M08.959 Juvenile arthritis, unspecified, unspecified hip
  • M08.95 Juvenile arthritis, unspecified, hip
  • M08.961 Juvenile arthritis, unspecified, right knee
  • M08.962 Juvenile arthritis, unspecified, left knee
  • M08.969 Juvenile arthritis, unspecified, unspecified knee
  • M08.96 Juvenile arthritis, unspecified, knee
  • M08.971 Juvenile arthritis, unspecified, right ankle and foot
  • M08.972 Juvenile arthritis, unspecified, left ankle and foot
  • M08.979 Juvenile arthritis, unspecified, unspecified ankle and foot
  • M08.97 Juvenile arthritis, unspecified, ankle and foot
  • M08.98 Juvenile arthritis, unspecified, vertebrae
  • M08.99 Juvenile arthritis, unspecified, multiple sites
  • M08.9 Juvenile arthritis, unspecified

SNOMED


  • 239796000 Juvenile chronic arthritis
  • 410795001 juvenile rheumatoid arthritis (disorder)
  • 410502007 juvenile idiopathic arthritis (disorder)
  • 74391003 Pauciarticular juvenile rheumatoid arthritis (disorder)
  • 239802003 juvenile psoriatic arthritis (disorder)
  • 410797009 Juvenile seronegative polyarthritis (disorder)
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