I. See Also

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. Rheumatoid Factor
    1. Variably positive, often normal
  2. Antinuclear Antibody
    1. May be associated with Uveitis (see above)
  3. Erythrocyte Sedimentation Rate (ESR)
    1. High ESR may suggest other diagnoses (e.g. infection)
  4. Complete Blood Count
    1. Abnormal in systemic presentation

VIII. Diagnosis (diagnosis of exclusion)

  1. Chronic arthritis of one or more joints for 6 weeks
  2. Other diagnoses excluded

IX. Management

  1. NSAIDs
  2. TNF agents

X. References

  1. Claudius and Behar in Majoewsky (2012) EM:RAP-C3 2(8): 2
  2. Prince (2010) BMJ 341:c6434 [PubMed] (or open in [QxMD Read])
  3. Schneider (2002) Rheum Dis Clin North Am 28:503-30 [PubMed] (or open in [QxMD Read])

Images: Related links to external sites (from Google)

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, 201796004, 156483006, 268052008, 86119004, 410794002, 287984007
English Chauffard-Still's disease, 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, STILL'S DISEASE, 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, Still's disease (disorder), juvenile onset Still's disease (diagnosis), juvenile Still's disease, juvenile onset Still's disease, STILL 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, Still's disease - juvenile R.A, Still's disease -RETIRED-, Still's disease - juvenile rheumatoid arthritis (disorder)
Dutch ziekte van Still, Still's disease
French Maladie de Still, Maladie de Still débutant dans l'enfance, Maladie de Still juvénile
German Still-Krankheit, Still-Krankheit, juvenile Form, Morbus Still, juvenile Form
Italian Malattia di Still
Portuguese Doença de Still, Doença de Still de Início Juvenil
Spanish Enfermedad de Still, enfermedad de Still - artritis reumatoide juvenil, enfermedad de Still - artritis reumatoide juvenil (trastorno), Still's disease, Still's disease - juvenile R.A, enfermedad de Still - RETIRADO - (concepto no activo), enfermedad de Still - RETIRADO -, enfermedad de Still, Enfermedad de Still de Inicio Juvenil
Japanese スチル病, スチルビョウ
Czech Stillova nemoc
Hungarian Still-kór

Ontology: Juvenile Chronic Polyarthritis (C0409667)

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

Ontology: Arthritis, Juvenile Rheumatoid (C0553662)

Definition (MEDLINEPLUS)

Juvenile rheumatoid arthritis (JRA) is a type of arthritis that happens in children age 16 or younger. It causes joint swelling, stiffness and sometimes reduced motion. It can affect any joint, and in some cases it can affect internal organs as well.

One early sign of JRA may be limping in the morning. Symptoms can come and go. Some children have just one or two flare-ups. Others have symptoms that never go away. JRA causes growth problems in some children.

No one knows exactly what causes JRA. Scientists do know it is an autoimmune disorder, which means your immune system, which normally helps your body fight infection, attacks your body's own tissues. Medicines and physical therapy can help maintain movement and reduce swelling and pain.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Definition (CSP) rheumatoid arthritis of children occurring in three major subtypes defined by the symptoms present during the first six months following onset: systemic onset (Still's Disease, Juvenile Onset), polyarticular onset, and pauciarticular onset; adult onset cases of Still's disease (Still's Disease, adult onset) are also known; only one subtype of juvenile rheumatoid arthritis (polyarticular onset, rheumatoid factor positive) clinically resembles adult rheumatoid arthritis and is considered its childhood equivalent.
Definition (MSH) Rheumatoid arthritis of children occurring in three major subtypes defined by the symptoms present during the first six months following onset: systemic-onset (Still's Disease, Juvenile-Onset), polyarticular-onset, and pauciarticular-onset. Adult-onset cases of Still's disease (STILL'S DISEASE, ADULT-ONSET) are also known. Only one subtype of juvenile rheumatoid arthritis (polyarticular-onset, rheumatoid factor-positive) clinically resembles adult rheumatoid arthritis and is considered its childhood equivalent.
Concepts Disease or Syndrome (T047)
MSH D001171
ICD10 M08 , M08.9, M08.0
SnomedCT 201808003, 201800005, 239796000, 86119004, 156483006, 268052008, 239801005, 410795001, 410502007, 410796000
English Rheumatoid arthritis, juvenile, Arthritis, Juvenile Chronic, Chronic Arthritis, Juvenile, Juvenile Chronic Arthritis, JUVENILE RHEUMATOID ARTHRITIS, Rheumatoid arthritis in children, ARTHRITIS, RHEUMATOID, JUVENILE, JRA, Rheumatoid Arthritis, Juvenile, Juvenile rheumatoid arthritis, NOS, JCA - Juvenile chronic arthrit, JRA-Juvenile rheumatoid arthr, Juvenile arthritis, unspecified, Juvenile rheumatoid arthr.NOS, Juvenile rheumatoid arthritis NOS, Juvenile seropositiv arthritis, juvenile rheumatoid arthritis, juvenile rheumatoid arthritis (diagnosis), Childhood arthritis, Juvenile RA, ARTHRITIS JUVENILE, ARTHRITIS RHEUMATOID JUVENILE, Juvenile rheumatoid arthritis NOS (disorder), Unspecified juvenile rheumatoid arthritis, Arthritis, Juvenile Rheumatoid [Disease/Finding], Arthritis, Juvenile Rheumatoid, rheumatoid arthritis in children, juvenile chronic arthritis, idiopathic juvenile arthritis, juvenile ra, children rheumatoid arthritis, jra, juvenile idiopathic arthritis, JCA - Juvenile chronic arthritis, Juvenile arthritis, Juvenile chronic arthritis, Juvenile chronic arthritis (disorder), juvenile; arthritis, rheumatoid, juvenile; arthritis, arthritis; juvenile, rheumatoid, arthritis; juvenile, arthritis; rheumatoid, juvenile, Juvenile rheumatoid a., JRA - Juvenile rheumatoid arthritis, Juvenile rheumatoid arthritis, Juvenile seropositive arthritis, Juvenile idiopathic arthritis, Juvenile rheumatoid arthritis (disorder), Juvenile chronic arthritis, polyarticular seropositive, Juvenile idiopathic arthritis (disorder), Juvenile idiopathic arthritis, polyarthritis, rheumatoid factor positive, Juvenile rheumatoid arthritis, seropositive, Juvenile seropositive polyarthritis (disorder), Juvenile seropositive polyarthritis, Juvenile Rheumatoid Arthritis, Arthritides, Juvenile Idiopathic, Arthritis, Juvenile Idiopathic, Idiopathic Arthritides, Juvenile, Idiopathic Arthritis, Juvenile, Juvenile Idiopathic Arthritides, Juvenile Idiopathic Arthritis, juvenile arthritis, Arthritis;juvenile
Portuguese ARTRITE REUMATOIDE JUVENIL, Artrite reumatóide juvenil, Artrite idiopática juvenil, Artrite Juvenil Idiopática, Artrite juvenil, Artrite Juvenil Crônica, Artrite Reumatóide Juvenil
Italian Artrite giovanile, Artrite idiopatica giovanile, Artrite reumatoide giovanile, Artrite idiopatica infantile, Artrite cronica infantile, Artrite reumatoide infantile
Dutch juveniele RA, juveniele idiopathische artritis, juveniele reumatoïde artritis, artritis; juveniel, reumatoïd, artritis; juveniel, artritis; reumatoïd, juveniel, juveniel; artritis, reumatoïd, juveniel; artritis, Juveniele artritis, niet gespecificeerd, juveniele artritis, Juveniele artritis, Artritis, juveniele chronische, Artritis, juveniele reumatoïde, Juveniele reumatoïde artritis, Rreumatoïde artritis, juveniele
French AR juvénile, Arthrite chronique juvénile, Polyarthrite chronique juvénile idiopathique, ARTHRITE CHRONIQUE JUVENILE, Arthrite juvénile idiopathique, AJI (Arthrite Juvénile Idiopathique), Polyarthrite chronique juvénile, Arthrite juvénile, Polyarthrite juvénile, Polyarthrite rhumatoïde juvénile
German juvenile idiopathische Arthritis, juvenile rheumatoide Arthritis, juvenile RA, Juvenile Arthritis, nicht naeher bezeichnet, Juvenile Arthritis, Juvenile chronische Polyarthritis, adulter Typ, juvenile Arthritis, Arthritis, juvenile rheumatoide, JRA, Juvenile chronische Arthritis, Juvenile rheumatoide Arthritis, Arthritis, juvenile chronische
Spanish Artritis idiopática juvenil, AR juvenil, Artritis reumatoide juvenil, artritis juvenil seropositiva, Juvenile rheumatoid arthritis, Juvenile rheumatoid a., artritis crónica juvenil (trastorno), artritis crónica juvenil, artritis juvenil, artritis reumatoide juvenil, SAI (trastorno), artritis reumatoide juvenil, SAI, artritis reumatoidea juvenil, SAI (trastorno), artritis reumatoidea juvenil, SAI, Artritis juvenil, artritis reumatoide juvenil (trastorno), artritis reumatoide juvenil, artritis idiopática juvenil (trastorno), artritis idiopática juvenil, poliartritis juvenil seropositiva (trastorno), poliartritis juvenil seropositiva, Artritis Juvenil Crónica, Artritis Reumatoide Juvenil, Artritis Juvenil Cronica, Artritis Idiopatica Juvenil, Artrítis Idiopática Juvenil
Japanese 若年性関節炎, 若年性特発性関節炎, ジャクネンセイトクハツセイカンセツエン, ジャクネンセイカンセツエン, ジャクネンセイカンセツリウマチ, 関節炎-若年性リウマチ性, 若年性関節リウマチ, スティル病, スチール病, スチル病, Still病-若年性, 関節炎-若年性慢性, 若年性慢性関節炎, 若年性スティル病, 若年性リウマチ性関節炎, 若年性Still病, スティル病-若年性, Still病, 関節リウマチ-若年性
Swedish Artrit, juvenil reumatoid
Czech artritida revmatoidní juvenilní, Juvenilní idiopatická artritida, Juvenilní revmatoidní artritida, Juvenilní artritida
Finnish Lastenreuma
Russian STILLA BOLEZN' IUNOSHESKAIA, ARTRIT IUNOSHESKII KHRONICHESKII, STILLA BOLEZN' IUNOSHESKAIA, ATAKA, STILLA BOLEZN' DETSKAIA, ARTRIT REVMATOIDNYI IUVENIL'NYI, АРТРИТ РЕВМАТОИДНЫЙ ЮВЕНИЛЬНЫЙ, АРТРИТ ЮНОШЕСКИЙ ХРОНИЧЕСКИЙ, СТИЛЛА БОЛЕЗНЬ ЮНОШЕСКАЯ, АТАКА, СТИЛЛА БОЛЕЗНЬ ДЕТСКАЯ, СТИЛЛА БОЛЕЗНЬ ЮНОШЕСКАЯ
Korean 청소년성 류마티스 관절염, 상세불명의 청소년성 관절염, 청소년성 관절염
Croatian ARTRITIS, REUMATOIDNI, JUVENILNI
Polish Gościec młodzieńczy przewlekły postępujący, Młodzieńcze reumatoidalne zapalenie stawów, Zapalenie stawów młodzieńcze idiopatyczne
Hungarian Juvenilis rheumatoid arthritis, Juvenilis arthritis, Juvenilis RA, Juvenilis idiopathiás arthritis

Ontology: Juvenile idiopathic arthritis, undifferentiated arthritis (C1444845)

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