PDF herunterladen
Kinder- und Jugendmedizin 2021; 21(05): 349-357
DOI: 10.1055/a-1584-5462
Schwerpunkt

Update – Systemische juvenile Arthritis

Update – Systemic juvenile arthritis
Toni Hospach∗
1   Zentrum für pädiatrische Rheumatologie am Klinikum Stuttgart, Pädiatrie 2 Olgahospital
,
Gerd Horneff∗
2   Kinderrheumazentrum Sankt Augustin, Zentrum für Allgemeine Pädiatrie und Neonatologie, Asklepios Klinik Sankt Augustin
› Institutsangaben
› Weitere Informationen
    Lizenzen und Reprints

ZUSAMMENFASSUNG

Die systemische juvenile idiopathische Arthritis nimmt unter den Kategorien der JIA eine Sonderstellung ein, klinisch aufgrund der obligat extraartikulären, systemischen Beteiligung, aber auch aufgrund der der vital bedrohlichen Komplikationen, Makrophagenaktivierungssyndrom und Lungenbeteiligung. Untersuchungen legen ein biphasisches Krankheitsgeschehen mit konsekutiven Eigenschaften einer autoinflammatorischen mit denen einer Autoimmunerkrankung nahe. Diese Erkenntnisse führten zu Therapieoptionen wie der IL-1- und IL-6-Blockade mit Biologika und zu Therapiealgorithmen, die zwischen früher Erkrankung und später Erkrankung unterscheiden, letztlich mit dem Ziel, der Nutzung eines „windows of opportunity“ zur Erreichung des Therapieziels der Remission.



Publikationsverlauf

Artikel online veröffentlicht:
18. Oktober 2021

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG,
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • Literatur

  • 1 Martini A, Ravelli A, Avcin T. et al Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus. J Rheumatol 2019; 46 (02) 190-197
    CrossrefPubMedGoogle Scholar
  • 2 Canna SW, Schulert GS, de Jesus A. et al Proceedings from the 2(nd) Next Gen Therapies for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome symposium held on October 3–4, 2019. Pediatr Rheumatol Online J 2020; 18 (Suppl. 01) 53
    CrossrefPubMedGoogle Scholar
  • 3 Gattorno M, Piccini A, Lasiglie D. et al The pattern of response to anti-interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis. Arthritis Rheum 2008; 58 (05) 1505-1515
    CrossrefPubMedGoogle Scholar
  • 4 Ter Haar NM, van Dijkhuizen EHP, Swart JF. et al Treatment to Target Using Recombinant Interleukin-1 Receptor Antagonist as First-Line Monotherapy in New-Onset Systemic Juvenile Idiopathic Arthritis: Results From a Five-Year Follow-Up Study. Arthritis Rheumatol 2019; 71 (07) 1163-1173
    CrossrefPubMedGoogle Scholar
  • 5 Pardeo M, Pires Marafon D, Insalaco A. et al Anakinra in Systemic Juvenile Idiopathic Arthritis: A Single-center Experience. J Rheumatol 2015; 42 (08) 1523-1527
    CrossrefPubMedGoogle Scholar
  • 6 Horneff G, Schulz AC, Klotsche J. et al Experience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry. Arthritis Res Ther 2017; 19 (01) 256
    CrossrefPubMedGoogle Scholar
  • 7 Saccomanno B, Tibaldi J, Minoia F. et al Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis. J Rheumatol 2019; 46 (04) 416-421
    CrossrefPubMedGoogle Scholar
  • 8 Pacharapakornpong T, Vallibhakara SA, Lerkvaleekul B, Vilaiyuk S. Comparisons of the outcomes between early and late tocilizumab treatment in systemic juvenile idiopathic arthritis. Rheumatol Int 2017; 37 (02) 251-255
    CrossrefPubMedGoogle Scholar
  • 9 Shimizu M, Nakagishi Y, Yachie A. Distinct subsets of patients with systemic juvenile idiopathic arthritis based on their cytokine profiles. Cytokine 2013; 61 (02) 345-348
    CrossrefPubMedGoogle Scholar
  • 10 Foell D, Wittkowski H, Vogl T, Roth J. S100 proteins expressed in phagocytes: a novel group of damage-associated molecular pattern molecules. J Leukoc Biol 2007; 81 (01) 28-37
    CrossrefPubMedGoogle Scholar
  • 11 Holzinger D, Frosch M, Kastrup A. et al The Toll-like receptor 4 agonist MRP8/14 protein complex is a sensitive indicator for disease activity and predicts relapses in systemic-onset juvenile idiopathic arthritis. Ann Rheum Dis 2012; 71 (06) 974-980
    CrossrefPubMedGoogle Scholar
  • 12 Föll D, Wittkowski H, Hinze C. Das Still-Syndrom als biphasische Erkrankung. Zeitschrift für Rheumatologie 2020; 79 (07) 639-648
    CrossrefPubMedGoogle Scholar
  • 13 Kessel C, Hedrich CM, Foell D. Innately Adaptive or Truly Autoimmune: Is There Something Unique About Systemic Juvenile Idiopathic Arthritis?. Arthritis Rheumatol 2020; 72 (02) 210-219
    CrossrefPubMedGoogle Scholar
  • 14 Mellins ED, Macaubas C, Grom AA. Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions. Nat Rev Rheumatol 2011; 07 (07) 416-426
    CrossrefPubMedGoogle Scholar
  • 15 Nigrovic PA, Mannion M, Prince FH. et al Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series. Arthritis Rheum 2011; 63 (02) 545-555
    CrossrefPubMedGoogle Scholar
  • 16 Behrens EM, Beukelman T, Gallo L. et al Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR). J Rheumatol 2008; 35 (02) 343-348
    Google Scholar
  • 17 Gurion R, Lehman TJ, Moorthy LN. Systemic arthritis in children: a review of clinical presentation and treatment. Int J Inflam 2012; 2012: 271569
    CrossrefPubMedGoogle Scholar
  • 18 Frosch M, Metze D, Foell D. et al Early activation of cutaneous vessels and epithelial cells is characteristic of acute systemic onset juvenile idiopathic arthritis. Exp Dermatol 2005; 14 (04) 259-265
    CrossrefPubMedGoogle Scholar
  • 19 Schneider R, Laxer RM. Systemic onset juvenile rheumatoid arthritis. Baillieres Clin Rheumatol 1998; 12 (02) 245-271
    CrossrefPubMedGoogle Scholar
  • 20 Behrens EM. Macrophage activation syndrome in rheumatic disease: what is the role of the antigen presenting cell?. Autoimmun Rev 2008; 07 (04) 305-308
    CrossrefPubMedGoogle Scholar
  • 21 Minoia F, Davi S, Horne A. et al Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol 2014; 66 (11) 3160-3169
    CrossrefPubMedGoogle Scholar
  • 22 Saper VE, Chen G, Deutsch GH. et al Emergent high fatality lung disease in systemic juvenile arthritis. Ann Rheum Dis 2019; 78 (12) 1722-1731
    CrossrefPubMedGoogle Scholar
  • 23 Dong S, Bout-Tabaku S, Texter K, Jaggi P. Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease. J Pediatr 2015; 166 (05) 1283-1288
    CrossrefPubMedGoogle Scholar
  • 24 Limenis E, Feldman BM, Achonu C. et al Proposed Core Set of Items for Measuring Disease Activity in Systemic Juvenile Idiopathic Arthritis. J Rheumatol 2018; 45 (01) 115-121
    CrossrefPubMedGoogle Scholar
  • 25 Foell D, Wittkowski H, Hammerschmidt I. et al Monitoring neutrophil activation in juvenile rheumatoid arthritis by S100A12 serum concentrations. Arthritis Rheum 2004; 50 (04) 1286-1295
    CrossrefPubMedGoogle Scholar
  • 26 Gohar F, McArdle A, Jones M. et al Molecular signature characterisation of different inflammatory phenotypes of systemic juvenile idiopathic arthritis. Ann Rheum Dis 2019; 78 (08) 1107-1113
    CrossrefPubMedGoogle Scholar
  • 27 Gohar F, Kessel C, Lavric M. et al Review of biomarkers in systemic juvenile idiopathic arthritis: helpful tools or just playing tricks?. Arthritis Res Ther 2016; 18: 163
    CrossrefPubMedGoogle Scholar
  • 28 Wittkowski H, Frosch M, Wulffraat N. et al S100A12 is a novel molecular marker differentiating systemic-onset juvenile idiopathic arthritis from other causes of fever of unknown origin. Arthritis Rheum 2008; 58 (12) 3924-3931
    CrossrefPubMedGoogle Scholar
  • 29 Rothmund F, Gerss J, Ruperto N. et al Validation of relapse risk biomarkers for routine use in patients with juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2014; 66 (06) 949-955
    CrossrefPubMedGoogle Scholar
  • 30 Aljaberi N, Tronconi E, Schulert G. et al The use of S100 proteins testing in juvenile idiopathic arthritis and autoinflammatory diseases in a pediatric clinical setting: a retrospective analysis. Pediatr Rheumatol Online J 2020; 18 (01) 7
    CrossrefPubMedGoogle Scholar
  • 31 Yasin S, Fall N, Brown RA. et al IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome. Rheumatology (Oxford) 2020; 59 (02) 361-366
    CrossrefPubMedGoogle Scholar
  • 32 Kudela H, Drynda S, Lux A. et al Comparative study of Interleukin-18 (IL-18) serum levels in adult onset Still’s disease (AOSD) and systemic onset juvenile idiopathic arthritis (sJIA) and its use as a biomarker for diagnosis and evaluation of disease activity. BMC Rheumatol 2019; 03: 4
    CrossrefPubMedGoogle Scholar
  • 33 Lee PY, Schulert GS, Canna SW. et al Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Ann Rheum Dis 2020; 79 (02) 225-231
    CrossrefPubMedGoogle Scholar
  • 34 Bracaglia C, de Graaf K, Pires Marafon D. et al Elevated circulating levels of interferon-gamma and interferon-gamma-induced chemokines characterise patients with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. Ann Rheum Dis 2017; 76 (01) 166-172
    CrossrefPubMedGoogle Scholar
  • 35 Eloseily EMA, Minoia F, Crayne CB. et al Ferritin to Erythrocyte Sedimentation Rate Ratio: Simple Measure to Identify Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. ACR Open Rheumatol 2019; 01 (06) 345-349
    CrossrefPubMedGoogle Scholar
  • 36 Tibaldi J, Pistorio A, Aldera E. et al Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis. Rheumatology (Oxford) 2020; 59 (11) 3505-3514
    CrossrefPubMedGoogle Scholar
  • 37 Vastert SJ, Jamilloux Y, Quartier P. et al Anakinra in children and adults with Still’s disease. Rheumatology (Oxford) 2019; 58 (Suppl. 06) vi9-vi22
    CrossrefPubMedGoogle Scholar
  • 38 Hinze CH, Holzinger D, Lainka E. et al Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany. Pediatr Rheumatol Online J 2018; 16 (01) 7
    CrossrefPubMedGoogle Scholar
  • 39 Hinze C, Kubasch AS, Berner R, Föll D. Systemische juvenile idiopathische Arthritis. Monatsschr Kinderheilkd 2018; 166 (10) 905-907
    CrossrefGoogle Scholar
  • 40 Nigrovic PA, Beukelman T, Tomlinson G. et al Bayesian comparative effectiveness study of four consensus treatment plans for initial management of systemic juvenile idiopathic arthritis: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST). Clin Trials 2018; 15 (03) 268-277
    CrossrefPubMedGoogle Scholar
  • 41 Kimura Y, Grevich S, Beukelman T. et al Pilot study comparing the Childhood Arthritis & Rheumatology Research Alliance (CARRA) systemic Juvenile Idiopathic Arthritis Consensus Treatment Plans. Pediatr Rheumatol Online J 2017; 15 (01) 23
    CrossrefPubMedGoogle Scholar
  • 42 Atemnkeng Ntam V, Klein A, Horneff G. Safety and efficacy of anakinra as first-line or second-line therapy for systemic onset juvenile idiopathic arthritis – data from the German BIKER registry. Expert Opin Drug Saf 2021; 20 (01) 93-100
    CrossrefPubMedGoogle Scholar
  • 43 Arthur VL, Shuldiner E, Remmers EF. et al IL1RN Variation Influences Both Disease Susceptibility and Response to Recombinant Human Interleukin-1 Receptor Antagonist Therapy in Systemic Juvenile Idiopathic Arthritis. Arthritis Rheumatol 2018; 70 (08) 1319-1330
    CrossrefPubMedGoogle Scholar
  • 44 Hinze C, Fuehner S, Kessel C. et al Impact of IL1RN Variants on Response to Interleukin-1 Blocking Therapy in Systemic Juvenile Idiopathic Arthritis. Arthritis Rheumatol 2020; 72 (03) 499-505
    CrossrefPubMedGoogle Scholar
  • 45 Brunner HI, Quartier P, Alexeeva E. et al Efficacy and Safety of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis With and Without Fever at Baseline: Results From an Open-Label, Active-Treatment Extension Study. Arthritis Rheumatol 2020; 72 (12) 2147-2158
    CrossrefPubMedGoogle Scholar
  • 46 Ruperto N, Brunner HI, Quartier P. et al Canakinumab in patients with systemic juvenile idiopathic arthritis and active systemic features: results from the 5-year long-term extension of the phase III pivotal trials. Ann Rheum Dis 2018; 77 (12) 1710-1719
    CrossrefPubMedGoogle Scholar
  • 47 Feist E, Quartier P, Fautrel B. et al Efficacy and safety of canakinumab in patients with Still’s disease: exposure-response analysis of pooled systemic juvenile idiopathic arthritis data by age groups. Clin Exp Rheumatol 2018; 36 (04) 668-675
    PubMedGoogle Scholar
  • 48 De Benedetti F, Brunner HI, Ruperto N. et al Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med 2012; 367 (25) 2385-2395
    CrossrefPubMedGoogle Scholar
  • 49 Mallalieu NL, Wimalasundera S, Hsu JC. et al Intravenous dosing of tocilizumab in patients younger than two years of age with systemic juvenile idiopathic arthritis: results from an open-label phase 1 clinical trial. Pediatr Rheumatol Online J 2019; 17 (01) 57
    CrossrefPubMedGoogle Scholar
  • 50 Malattia C, Ruperto N, Pederzoli S. et al Tocilizumab may slow radiographic progression in patients with systemic or polyarticular-course juvenile idiopathic arthritis: post hoc radiographic analysis from two randomized controlled trials. Arthritis Res Ther 2020; 22 (01) 211
    CrossrefPubMedGoogle Scholar
  • 51 Pardeo M, Wang J, Ruperto N. et al Neutropenia During Tocilizumab Treatment Is Not Associated with Infection Risk in Systemic or Polyarticular-course Juvenile Idiopathic Arthritis. J Rheumatol 2019; 46 (09) 1117-1126
    CrossrefPubMedGoogle Scholar
  • 52 Gupta A, Bagri NK, Tripathy SK. et al Successful use of tocilizumab in amyloidosis secondary to systemic juvenile idiopathic arthritis. Rheumatol Int 2020; 40 (01) 153-159
    CrossrefPubMedGoogle Scholar
  • 53 Kiltz U, Kiefer D, Braun J. et al Prolonged treatment with Tadekinig alfa in adult-onset Still’s disease. Ann Rheum Dis 2020; 79 (01) e10
    CrossrefPubMedGoogle Scholar
  • 54 Gabay C, Fautrel B, Rech J. et al Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still’s disease. Ann Rheum Dis 2018; 77 (06) 840-847
    PubMedGoogle Scholar
  • 55 Bader-Meunier B, Hadchouel A, Berteloot L. et al Effectiveness and safety of ruxolitinib for the treatment of refractory systemic idiopathic juvenile arthritis like associated with interstitial lung disease: a case report. 2020: 216983
    Google Scholar
  • 56 Huang Z, Lee PY, Yao X, Zheng S, Li T. Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis. Pediatrics 2019; 143 (05) e20182845
    CrossrefPubMedGoogle Scholar
  • 57 Hu Q, Wang M, Jia J. et al Tofacitinib in refractory adult-onset Still’s disease: 14 cases from a single centre in China. Ann Rheum Dis 2020; 79 (06) 842-844
    CrossrefPubMedGoogle Scholar
  • 58 Wallace CA, Levinson JE. Juvenile rheumatoid arthritis: outcome and treatment for the 1990 s. Rheum Dis Clin North Am 1991; 17 (04) 891-905
    CrossrefPubMedGoogle Scholar
  • 59 Minden K, Kiessling U, Listing J. et al Prognosis of patients with juvenile chronic arthritis and juvenile spondyloarthropathy. J Rheumatol 2000; 27 (09) 2256-2263
    Google Scholar
  • 60 Oen K, Malleson PN, Cabral DA. et al Disease course and outcome of juvenile rheumatoid arthritis in a multicenter cohort. J Rheumatol 2002; 29 (09) 1989-1999
    Google Scholar
  • 61 Wallace CA, Huang B, Bandeira M. et al Patterns of clinical remission in select categories of juvenile idiopathic arthritis. Arthritis Rheum 2005; 52 (11) 3554-3562
    CrossrefPubMedGoogle Scholar