HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Special course of primary chronic polyarthritis (rheumatoid arthritis) in childhood (see Juvenile idiopathic arthritis) with lymphadenopathy, re- and intermittent fever, carditis, liver and spleen enlargement, anemia, and recurrent polymorphic exanthema and high ESR.
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Occurrence/EpidemiologyThis section has been translated automatically.
EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
- Arthritis/polyarthritis: Arthralgias, arthritides with bilateral swelling of the wrists, knees and ankles, and myalgias. Symmetrical polyarthritis of large and small joints developing slowly over months. In some patients, the polyarthritis becomes chronic, leading to loss of function and joint destruction.
- Intermittent fevers up to 40°C with 1-2 spikes/day that may persist for weeks. The diagnosis is based on these symptoms being accompanied by 1 or more of the following criteria (1-4):
- Volatile, urticarial, morbilliform, or scarlatiniform exanthema (high recurrence) on the extremities, trunk, and face.
- Generalized nonpainful lymphadenopathy (25%).
- Hepatosplenomegaly (10-15%)
- Pleurisy and pericarditis (7-20%).
- Myocarditis is feared, which can lead to heart failure.
- Ocular changes: iridocyclitis, corneal degeneration, later cataract.
LaboratoryThis section has been translated automatically.
BSG strongly accelerated
CRP strongly increased
Leukocytosis up to 50,000/μl
Anemia (children complain of poor general condition with fatigue, lassitude, loss of appetite, among other symptoms),
Elevation of IL-18 and MRP8/14 (S100),
A check of the ferritin level ensures the timely detection of an increased risk of MAS/> 1,000 ng/ml (MAS=macrophage activation syndrome).
Important: no antinuclear antibodies or rheumatoid factors are detectable in Still syndrome.
DiagnosisThis section has been translated automatically.
Complication(s)This section has been translated automatically.
Growth retardation. A severe complication of the systemic form can be marked anemia.
In the context of Still's syndrome, complications such as macrophage activation syndrome (MAS), which occurs in about 10% of affected SJIA patients, and amyloidosis may occur in all patient groups. Secondary amyloidosis is one of the most serious long-term complications (about 4%). It can lead to renal failure with marked proteinuria and results in a high lethality rate.
TherapyThis section has been translated automatically.
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.
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 (16)Arthritis atypical infantile rheumatoid; Arthritis chronic juvenile seronegative; Arthritis chronic systemic juvenile; Breastfeeding syndrome adultes; Chauffard-raymond syndrome; Cinca syndrome; Dermatitis-arthritis syndromes; Erythema multiforme rheumatoides; Eye diseases, skin changes; Juvenile Idiopathic Arthritis; ... Show all
Outgoing links (9)Amyloidosis systemic (overview); Autoinflammatory syndromes (overview); Breastfeeding syndrome adultes; Cataract; Ferritin; Interleukin-18; Juvenile Idiopathic Arthritis; Rash; Rheumatoid arthritis and skin manifestations;
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