The 4th NextGen Therapies for SJIA and MAS: Part 1 the Elephant in the Room: Diagnostic/classification Criteria for Systemic Juvenile Idiopathic Arthritis and Adult-onset Still's Disease

Overview

Journal Pediatr Rheumatol Online J

Publisher Biomed Central

Specialty Pediatrics

Date 2024 Jan 5

PMID 38183114

Authors

Peter A Nigrovic

Fabrizio De Benedetti

Yukiko Kimura

Daniel J Lovell

Sebastiaan J Vastert

Affiliations

Soon will be listed here.

Abstract

Currently, the criteria used to classify patients with SJIA are different from those used for AOSD. However, it has been recognized that the existing terms are too narrow, subdividing the Still's population unnecessarily between pediatric-onset and adult-onset disease and excluding an appreciable group of children in whom overt arthritis is delayed or absent. Government regulators and insurers rely upon the guidance of subject experts to provide disease definitions, and when these definitions are flawed, to provide new and better ones. The classification session at the NextGen 2022 conference helped to serve this purpose, establishing the need for a revised definitional system that transcends the fault lines that remain in existing definitions.

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References

Petty R, Southwood T, Manners P, Baum J, Glass D, Goldenberg J . International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004; 31(2):390-2. View

Jamilloux Y, Georgin-Lavialle S, Seve P, Belot A, Fautrel B . [It is time to reconcile systemic juvenile idiopathic arthritis and adult-onset Still's disease]. Rev Med Interne. 2019; 40(10):635-636. DOI: 10.1016/j.revmed.2019.06.001. View

Martini A, Ravelli A, Avcin T, Beresford M, Burgos-Vargas R, Cuttica R . Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus. J Rheumatol. 2018; 46(2):190-197. DOI: 10.3899/jrheum.180168. View

Ter Haar N, Tak T, Mokry M, Scholman R, Meerding J, de Jager W . Reversal of Sepsis-Like Features of Neutrophils by Interleukin-1 Blockade in Patients With Systemic-Onset Juvenile Idiopathic Arthritis. Arthritis Rheumatol. 2018; 70(6):943-956. DOI: 10.1002/art.40442. View

Morgan DeWitt E, Kimura Y, Beukelman T, Nigrovic P, Onel K, Prahalad S . Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2012; 64(7):1001-10. PMC: 3368104. DOI: 10.1002/acr.21625. View

Cader M, Boroviak K, Zhang Q, Assadi G, Kempster S, Sewell G . C13orf31 (FAMIN) is a central regulator of immunometabolic function. Nat Immunol. 2016; 17(9):1046-56. PMC: 6581540. DOI: 10.1038/ni.3532. View

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, Iwaz J, Ninet J, Durieu I . Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014; 93(2):91-99. PMC: 4616309. DOI: 10.1097/MD.0000000000000021. View

Prieur A, Bremard-Oury C, Griscelli C, MOZZICONACCI P . [Prognosis of the systemic forms of juvenile chronic arthritis. Apropos of 100 cases]. Arch Fr Pediatr. 1984; 41(2):91-7. View

Nigrovic P, Raychaudhuri S, Thompson S . Review: Genetics and the Classification of Arthritis in Adults and Children. Arthritis Rheumatol. 2017; 70(1):7-17. PMC: 5805142. DOI: 10.1002/art.40350. View

10.

Grom A, Villanueva J, Lee S, Goldmuntz E, Passo M, Filipovich A . Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome. J Pediatr. 2003; 142(3):292-6. DOI: 10.1067/mpd.2003.110. View

11.

Ter Haar N, van Dijkhuizen E, Swart J, Royen-Kerkhof A, El Idrissi A, Leek A . 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(7):1163-1173. PMC: 6617757. DOI: 10.1002/art.40865. View

12.

Shimizu M, Nakagishi Y, Yachie A . Distinct subsets of patients with systemic juvenile idiopathic arthritis based on their cytokine profiles. Cytokine. 2013; 61(2):345-8. DOI: 10.1016/j.cyto.2012.11.025. View

13.

Case S, Nigrovic P . Implications of Evolving Disease Classification for Drug Approval in Juvenile Idiopathic Arthritis. Paediatr Drugs. 2022; 24(3):185-191. PMC: 10389810. DOI: 10.1007/s40272-022-00496-0. View

14.

Gattorno M, Piccini A, Lasiglie D, Tassi S, Brisca G, Carta S . The pattern of response to anti-interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis. Arthritis Rheum. 2008; 58(5):1505-15. DOI: 10.1002/art.23437. View

15.

Bywaters E . Still's disease in the adult. Ann Rheum Dis. 1971; 30(2):121-33. PMC: 1005739. DOI: 10.1136/ard.30.2.121. View

16.

Russo R, Katsicas M . Patients with very early-onset systemic juvenile idiopathic arthritis exhibit more inflammatory features and a worse outcome. J Rheumatol. 2013; 40(3):329-34. DOI: 10.3899/jrheum.120386. View

17.

ANSELL B . Heberden Oration, 1977. Chronic arthritis in childhood. Ann Rheum Dis. 1978; 37(2):107-20. PMC: 1001175. DOI: 10.1136/ard.37.2.107. View

18.

Still G . On a Form of Chronic Joint Disease in Children. Med Chir Trans. 2010; 80:47-60.9. PMC: 2036674. View

19.

ANSELL B, Bywaters E . Prognosis in Still's disease. Bull Rheum Dis. 1959; 9(9):189-92. View

20.

Nigrovic P, Colbert R, Holers V, Ozen S, Ruperto N, Thompson S . Biological classification of childhood arthritis: roadmap to a molecular nomenclature. Nat Rev Rheumatol. 2021; 17(5):257-269. PMC: 10355214. DOI: 10.1038/s41584-021-00590-6. View