Arthritis atypical infantile rheumatoid; Arthritis chronic juvenile seronegative; Arthritis chronic systemic juvenile; atypical infantile rheumatoid arthritis; Chauffard-Ramon syndrome; Chauffard-Raymond Syndrome; Chauffard-Still Syndrome; chronic arthritis; juvenile; leucocytotic arthritis (Bessau); Rheumatoid arthritis atypical infantile; seronegative; Still's disease; systemic; Triple Syndrome
HistoryThis section has been translated automatically.
Cornil, 1864; Still, 1896; Chauffard, 1896
DefinitionThis section has been translated automatically.
Special course of primary chronic polyarthritis (rheumatoid arthritis) in childhood with lymphadenopathy, re- and intermittent fever, carditis, enlargement of liver and spleen, anaemia and recurrent polymorphic exanthema.
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Occurrence/EpidemiologyThis section has been translated automatically.
The most frequent disease of the rheumatic form in childhood. Incidence: 5-20/100.000 children/year; prevalence: 30-200/100.000 children.
EtiopathogenesisThis section has been translated automatically.
Unknown. Disturbances in immune regulation as well as viral and bacterial infections are discussed.
ManifestationThis section has been translated automatically.
Start of the disease in the 1st-4th year of life (start of the disease in adulthood is possible).
Clinical featuresThis section has been translated automatically.
- Arthritis/polyarthritis: Arthralgias, arthritides with bilateral swelling of the wrists, knees and ankles and myalgia. Symmetrical polyarthritis of large and small joints that develops slowly over months. In some patients, polyarthritis becomes chronic, leading to loss of function and joint destruction.
- Intermittent fever attacks up to 40°C with 1-2 peaks/day, which can persist for weeks. These symptoms are diagnosed if they are accompanied by 1 or more of the following criteria (1-4):
- Volatile, urticarial, morbilliform or scarlatiniform exanthema (high recurrence rate) of the extremities, trunk and face.
- Generalized, non-painful lymphadenopathy (25%)
- Hepatosplenomegaly (10-15%)
- Pleuritis and pericarditis (7-20%).
- Feared is myocarditis, which can lead to heart failure.
- Eye changes: iridocyclitis, corneal degeneration, later cataract.
LaboratoryThis section has been translated automatically.
BSG acceleration; rheumatoid factor: negative; increase of α1- and α2-globulins in serum electrophoresis; in case of prolonged course of disease IgG proliferation; hypochromic anaemia; leucocytosis (neutrophilia); thrombocytosis.
DiagnosisThis section has been translated automatically.
Radiologically, osteoporosis close to the joints and periosteal reactions show up as early changes. In advanced cases, evidence of marginal usuria and joint space narrowing. Early closure of the epiphyseal joints.
Complication(s)This section has been translated automatically.
Growth retardation. A severe complication of the systemic form can be pronounced anemia. One of the most serious long-term complications is secondary amyloidosis (about 4%), which leads to renal insufficiency with pronounced proteinuria and results in a high mortality rate.
TherapyThis section has been translated automatically.
Non-steroidal anti-inflammatory drugs, possibly corticoids or basic therapeutics, corresponding to primary chronic polyarthritis. Physiotherapy.
Progression/forecastThis section has been translated automatically.
- Progression in phases, possible growth arrest after reaching adulthood.
- Prognostic factors associated with severe disease progression:
- manifestation of the systemic form before or at the age of 6 months
- Female sex
- Rheumatoid factor detection: positive
- Persistent morning stiffness
- Subcutaneous nodules
- ANCA detection: positive
- Early infestation of the small wrists and ankles
- Suddenly appearing skin erosions.
Note(s)This section has been translated automatically.
The (infantile) Stiil Syndrome, like the adult Stiil Syndrome, belongs to the extended circle of the so-called autoinflammatory syndromes.
LiteratureThis section has been translated automatically.
- Chauffard A, Ramon (1896) Des adénopathies dans le rhumatisme chronique infectieux. Rev méd 16: 345
- Colebatch-Bourn AN et al (2015) EULAR-PReS points to consider for the use of imaging in the diagnosis and management of juvenile idiopathic arthritis in clinical practice. Ann Rheum Dis doi: 10.1136/annrheumdis-2015-207892
- Cornil V (1864) Mémoire sur les coincidences pathologiques du rhumatisme articulaire chroniques. Comptes rendus des séances et mémoires de la Société de biologie (Paris) 1: 3-25
- Dong S et al (2015) Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease. J Pediatr 166:1283-1288
- Golsch S, Engst R, Borelli S (1992) The Still Syndrome from a dermatological point of view. Z Hautkr 67: 394-396
- Hoeger PH et al (2000) Neonatal onset of rash in Still's disease. J Pediatr 137: 128-131
- Husni ME et al (2002) Etanercept in the treatment of adult patients with Still's disease. Arthritis Rheum 46: 1171-1176
- Ilowite NT (2002) Current treatment of juvenile rheumatoid arthritis. Pediatrics 109: 109-115
- Minden K et al (2002) Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum 46: 2392-2401
- Quartier P et al (2003) Efficacy of etanercept for the treatment of juvenile idiopathic arthritis according to the onset type. Arthritis Rheum 48: 1093-1101
- Schanberg LE (2003) Daily pain and symptoms in children with polyarticular arthritis. Arthritis Rheum 48: 1390-1397
- Still GF (1896) A special form of joint disease met with in children. Doctoral dissertation, Cambridge (UK)
- Still GF (1897) On a form of chronic joint disease in children. Med Chir Transact 80: 47
Incoming links (12)Arthritis atypical infantile rheumatoid; Arthritis chronic juvenile seronegative; Arthritis chronic systemic juvenile; Chauffard-raymond syndrome; Cinca syndrome; Dermatitis-arthritis syndromes; Dermatitis rheumatoid neutrophils; Erythema multiforme rheumatoides; Eye diseases, skin changes; Kikuchi fujimoto syndrome; ... Show all
Outgoing links (5)Amyloidosis systemic (overview); Autoinflammatory syndromes (overview); Cataract; Rash; Rheumatoid arthritis (ra);
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