II. Pathophysiology

  1. Rheumatoid disease in children

III. Types

  1. Pauciarticular (4 or fewer joints involved)
    1. Age at presentation <8 years
    2. Involves large joints and is typically asymmetric
    3. Boys may develop Sacroiliitis
    4. Iridocyclitis may be present
  2. Polyarticular (>4 joints involved)
    1. Age at presentation 1-6 or 11-16 years
    2. Involves small joints with symmetric involvement
    3. Rheumatoid Factor may be positive
      1. Positive Rheumatoid Factor confers similar presentation to Rheumatoid Arthritis in adults
  3. Systemic (Still's Disease)
    1. High daily Relapsing Fever >39 degrees persists over 2 weeks (most distinguishes this type from others)
    2. Polyarticular involvement with >4 joints involved
    3. Hepatomegaly and Splenomegaly
    4. Lymphadenopathy
    5. Pleural Effusion or Pericardial Effusion may be present
    6. Recurrent evanescent rash
      1. Faint salmon colored rash over trunk, palms and soles

IV. Symptoms

  1. Pediatric Limp (most common presentation)
  2. Pain is minimal compared to inflammatory signs
    1. Increasing pain has Negative Predictive Value for RA

V. Signs

  1. Similar to adult form of Rheumatoid Arthritis
    1. Joint Swelling with warmth (not red and hot)
  2. Differences from Adult Rheumatoid Arthritis
    1. Higher fever
    2. Rheumatoid Nodules are rare
    3. Pericarditis and valvulitis are more common
    4. Lymphadenopathy and Hepatosplenomegaly are common
    5. Arthritis interferes with bone growth
      1. Example: underdeveloped Mandible
    6. Uveitis most common juvenile RA eye disorder
      1. Occurs in up to 50% of Pauciarticular juvenile RA
        1. Highest risk if ANA positive and recent onset
      2. Asymptomatic in up to 80% of cases
      3. Delayed diagnosis risks Cataracts, Glaucoma, blind
      4. Ophthalmology should follow all with juvenile RA

VII. Labs (often non-diagnostic)

  1. Precautions
    1. Rheumatoid Factor and Antinuclear Antibody are negative in more than 80% of juvenile RA
  2. Rheumatoid Factor
    1. Variably positive, often normal
  3. Antinuclear Antibody
    1. May be associated with Uveitis (see above)
  4. Erythrocyte Sedimentation Rate (ESR)
    1. High ESR may suggest other diagnoses (e.g. infection)
  5. C-Reactive Protein (CRP)
  6. Complete Blood Count
    1. Abnormal in systemic presentation

VIII. Imaging

  1. XRay affected joints

IX. Diagnosis (diagnosis of exclusion)

  1. Chronic Arthritis of one or more joints for 6 weeks
  2. Other diagnoses (including infection, Leukemia) excluded

X. Management: General

  1. NSAIDs
  2. Methotrexate
  3. CD-20 Cytolytic Monoclonal Antibody (e.g. Rituxamab)

XI. Management: Exacerbation

  1. Obtain labs (normal findings do not exclude flare)
    1. Complete Blood Count with differential
    2. Erythrocyte Sedimentation Rate
    3. C-Reactive Protein (CRP)
  2. Associated serious conditions
    1. Infection
      1. CD-20 Cytolytic Monoclonal Antibody (e.g. Rituxamab) result in Immunocompromised state
      2. Labs as above and consider Lactic Acid and Blood Cultures
      3. Exclude serious infection and treat obvious causes
      4. Stop Monoclonal Antibody or other immunosuppressants until infection resolves
    2. Septic Joint
      1. Febrile patient with red, hot joint and increased CRP and ESR
      2. Joint Aspiration if unclear diagnosis
    3. Macrophage activation syndrome
      1. Cytokine "storm" with similar appearance to Sepsis, with risk of DIC, Acute Renal Failure, Pancytopenia
      2. Treated with high dose pulse Corticosteroids and supportive care
  3. Symptomatic flare management
    1. NSAIDs
      1. First-line treatment for mild to moderate Arthritis symptoms if not taken consistently
    2. Corticosteroids
      1. Second-line treatment for moderate to severe Arthritis symptoms, especially if NSAID refractory
      2. Prednisolone 1-2 mg/kg orally divided twice daily for 3-5 days
        1. Consider initial dose of Methylprednisolone 1-2 mg/kg IV if in Emergency Department
    3. Hospitalization indications
      1. Inability to ambulate
      2. Concurrent serious infection

XII. Prognosis

  1. Immunomodulating agents significantly improve longterm outcomes with less chronic Disability

XIII. References

  1. Claudius, Behar and Chang in Herbert (2016) EM:RAP 16(1): 11-2
  2. Claudius and Behar in Herbert (2012) EM:RAP-C3 2(8): 2
  3. Gowdie (2012) Pediatr Clin North Am 59(2):301-27 +PMID:22560572 [PubMed]
  4. Prince (2010) BMJ 341:c6434 +PMID:21131338 [PubMed]
  5. Schneider (2002) Rheum Dis Clin North Am 28:503-30 [PubMed]

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Related Studies

Ontology: Juvenile-Onset Still Disease (C0087031)

Definition (NCI) An inflammatory disorder most often affecting children. It is characterized by the presence of arthritis, salmon-colored rash, spiking fevers, fatigue, and sore throats.
Concepts Disease or Syndrome (T047)
MSH D001171
ICD10 M08.2
SnomedCT 123246003, 156483006, 268052008, 287984007, 201796004, 86119004, 410794002
English Juvenile Onset Still Disease, Juvenile Onset Stills Disease, Juvenile-Onset Still's Disease, Juvenile-Onset Stills Disease, Still Disease, Juvenile Onset, Still Disease, Juvenile-Onset, Still's Disease, Juvenile Onset, Still's Disease, Juvenile-Onset, Stills Disease, Juvenile-Onset, Juvenile-Onset Still Disease, STILL DISEASE JUVENILE ONSET, JUVENILE ONSET STILL DIS, JUVENILE ONSET STILLS DIS, STILLS DIS JUVENILE ONSET, Still's dis - juv rheum arthr, Still's disease - juvenile rheumatoid arthritis, juvenile onset Still's disease (diagnosis), juvenile Still's disease, juvenile onset Still's disease, Still Disease, Still's Disease, Still's disease NOS, disease still, still's disease, systemic onset jra, disease still's, disease stills, still disease, Still's disease - juvenile R.A, Still's disease (disorder), Still's disease - juvenile rheumatoid arthritis (disorder), Still's disease, Still's disease -RETIRED-
French Maladie de Still débutant dans l'enfance, Maladie de Still
German Still-Krankheit, juvenile Form, Still-Krankheit, Morbus Still, juvenile Form
Czech Stillova choroba dětí, Stillova choroba, začínající v dětství, Stillova nemoc
Norwegian Stills sykdom, Stills sykdom hos barn, Stills sykdom med debut hos barn
Dutch ziekte van Still, Still's disease
Hungarian Still-kór
Italian Malattia di Still, Malattia di Still a esordio giovanile, Morbo di Still
Japanese スチルビョウ, スチル病
Portuguese Doença de Still, Doença de Still de Início Juvenil
Spanish Enfermedad de Still, enfermedad de Still - artritis reumatoide juvenil, enfermedad de Still - RETIRADO -, enfermedad de Still - RETIRADO - (concepto no activo), enfermedad de Still - artritis reumatoide juvenil (trastorno), enfermedad de Still, Enfermedad de Still de Inicio Juvenil

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Concepts Disease or Syndrome (T047)
ICD9 714.3
SnomedCT 201803007, 7441009
English Polyart onset juv chr arthrit, Polyarticular juven arthritis, Polyarticular juvenile arthritis, Polyarticular onset juvenile chronic arthritis, Juvenile Chronic Polyarthritis, Juvenile chronic polyarthritis, Polyarticular onset juvenile chronic arthritis (disorder), Juvenile chronic polyarthritis (disorder), Juvenile chronic polyarthritis, NOS
Czech Juvenilní chronická polyartritida
Dutch juveniele chronische polyartritis
French Polyarthrite chronique juvénile
German juvenile chronische Polyarthritis
Hungarian Juvenilis chronikus polyarthritis
Italian Poliartrite cronica giovanile
Japanese ジャクネンセイマンセイタハツカンセツエン, 若年性慢性多発関節炎
Portuguese Poliartrite crónica juvenil
Spanish Poliartritis crónica juvenil, poliartritis crónica juvenil, poliartritis crónica juvenil (trastorno)

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Concepts Disease or Syndrome (T047)
SnomedCT 410802003
English Juvenile idiopathic arthritis, undifferentiated arthritis (disorder), Juvenile idiopathic arthritis, undifferentiated arthritis
Spanish artritis idiopática juvenil, artritis indiferenciada (trastorno), artritis idiopática juvenil, artritis indiferenciada