Systemic juvenile idiopathic arthritis (Still syndrome) M08.29

Special form of primary chronic polyarthritis (rheumatoid arthritis) in children with lymphadenopathy, recurrent and intermittent high fever, carditis, liver and spleen enlargement, anemia and a recurrent salmon-colored, polymorphic exanthema, leukocytosis, pleuritis, pericraditis and high ESR. Initially, joint involvement is usually in the background (see alsoILAR classification)

Onset of the disease in the 1st-4th year of life (onset of the disease in adulthood is possible, see below). Adult Still's syndrome/AOSD).

• 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):
  1. Volatile, urticarial, morbilliform, or scarlatiniform exanthema (high recurrence) on the extremities, trunk, and face.
  2. Generalized nonpainful lymphadenopathy (25%).
  3. Hepatosplenomegaly (10-15%)
  4. Pleurisy and pericarditis (7-20%).
• Myocarditis is feared, which can lead to heart failure.
• Ocular changes: iridocyclitis, corneal degeneration, later cataract.

BSG strongly accelerated

CRP strongly increased

Leukocytosis up to 50,000/μl

Thrombocytosis

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.

Growth retardation. A severe complication of the systemic form can be pronounced anemia.

In the context of Still's syndrome, complications such as macrophage activation syndrome (MAS), which occurs in around 10% of affected SJIA patients, and amyloidosis can occur in all patient groups. Secondary amyloidosis is one of the most serious long-term complications (around 4%). It can lead to renal insufficiency with pronounced proteinuria and results in a high mortality rate.

• 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
• Tendosynovitis
• Subcutaneous nodules
• ANCA detection: positive
• Early infestation of the small wrists and ankles
• Suddenly appearing skin erosions.

Like adult Still's syndrome, (infantile) Still's syndrome belongs to the extended group of so-called autoinflammatory syndromes.

1. Chauffard A, Ramon (1896) Des adénopathies dans le rhumatisme chronique infectieux. Rev méd 16: 345
2. 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
3. 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
4. Dong S et al (2015) Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease. J Pediatr 166:1283-1288
5. Golsch S, Engst R, Borelli S (1992) The Still Syndrome from a dermatological point of view. Z Hautkr 67: 394-396
6. Hoeger PH et al (2000) Neonatal onset of rash in Still's disease. J Pediatr 137: 128-131
7. Husni ME et al (2002) Etanercept in the treatment of adult patients with Still's disease. Arthritis Rheum 46: 1171-1176
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9. Minden K et al (2002) Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum 46: 2392-2401
10. 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
11. Schanberg LE (2003) Daily pain and symptoms in children with polyarticular arthritis. Arthritis Rheum 48: 1390-1397
12. Still GF (1896) A special form of joint disease met with in children. Doctoral dissertation, Cambridge (UK)
13. Still GF (1897) On a form of chronic joint disease in children. Med Chir Transact 80: 47