Login Register Cart 0
Generic placeholder image

Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Back
Journal Home About Journal Editorial Board Journal Insight Current Issue Volumes/Issues
Subscribe
Cross-Sectional Study

Adult-onset and Juvenile-onset Still’s Disease: A Comparative Study of Both Sides

Author(s): Ben Tekaya Aicha*, Hannech Emna, Saidane Olfa, Bouden Selma, Rouached Leila, Tekaya Rawda, Mahmoud Ines, Abdelmoula Slim and Abdelmoula Leila

Volume 19, Issue 2, 2023

Published on: 21 July, 2022

Page: [235 - 241] Pages: 7

DOI: 10.2174/1573397118666220617101447

Price: $65

Abstract

Background: Adult-onset Still’s disease and systemic-onset juvenile idiopathic arthritis constitute two sides of the same continuum disease. We aimed to investigate the similarities and differences between those diseases.

Methods: We conducted a retrospective study including adult patients affected by still’s disease, attending the rheumatology department and patients affected by systemic-onset juvenile idiopathic arthritis attending the pediatric department. We recorded clinical and radiological findings, different therapeutic regimens, and disease patterns.

Results: There were 8 adult patients (6 females and 2 males) and 8 juvenile patients (4 females and 4 males). The classical triad of spiking fever, arthritis, and evanescent skin rash was the first clinical presentation observed in 4 adult patients and in 2 juvenile patients. Arthritis was noted in 8 adult patients versus 6 juvenile patients. Joint deformities were seen in adult patients. Non-steroid antiinflammatory drugs and corticosteroids were the most prescribed molecules. csDMARDs and bDMARDs were used in second-line therapy only for adult patients. The monocyclic course was predominant in juvenile patients and the polycyclic course in adult patients. The chronic course was observed only in two adult patients. Remission was noted in 5 adult patients and 6 juvenile patients. There were no significant differences between the two groups regarding clinical findings, different therapeutic regimens, and disease patterns.

Conclusion: From the findings of our study, it seems that AOSD and sJIA are the same syndrome continuum expressed in different hosts. This hypothesis is supported by clinical course, molecule evidence, cytokine profile, and treatment response.

Keywords: Adult-onset still’s disease, arthritis juvenile, comparative study, arthritis, interleukin 1 receptor antagonist protein, tumor necrosis factor inhibitors.

« Previous
Graphical Abstract

[1]
Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P. Adult-onset still’s disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum 2021; 51(4): 858-74.
[http://dx.doi.org/10.1016/j.semarthrit.2021.06.004] [PMID: 34175791]
[2]
Wakai K, Ohta A, Tamakoshi A, et al. Estimated prevalence and incidence of adult Still’s disease: Findings by a nationwide epidemiological survey in Japan. J Epidemiol 1997; 7(4): 221-5.
[http://dx.doi.org/10.2188/jea.7.221] [PMID: 9465547]
[3]
Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: Second revision, Edmonton, 2001. J Rheumatol 2004; 31(2): 390-2.
[PMID: 14760812]
[4]
Theodoropoulou K, Georgin-Lavialle S. Systemic juvenile onset idiopathic arthritis and adult onset still disease. Rev Med Suisse 2018; 14(594): 372-7.
[http://dx.doi.org/10.53738/REVMED.2018.14.594.0372] [PMID: 29461751]
[5]
Zeng T, Zou YQ, Wu MF, Yang CD. Clinical features and prognosis of adult-onset still’s disease: 61 cases from China. J Rheumatol 2009; 36(5): 1026-31.
[http://dx.doi.org/10.3899/jrheum.080365] [PMID: 19273456]
[6]
Patra S, Santosh T, Goel G, Rupla R. Dermato-pathologic clues to diagnosis of adult onset still disease: A case report. Indian Dermatol Online J 2021; 12(5): 741-4.
[http://dx.doi.org/10.4103/idoj.IDOJ_383_20] [PMID: 34667762]
[7]
Jamilloux Y, Gerfaud-Valentin M, Martinon F, Belot A, Henry T, Sève P. Pathogenesis of adult-onset Still’s disease: New insights from the juvenile counterpart. Immunol Res 2015; 61(1-2): 53-62.
[http://dx.doi.org/10.1007/s12026-014-8561-9] [PMID: 25388963]
[8]
Inoue N, Shimizu M, Tsunoda S, Kawano M, Matsumura M, Yachie A. Cytokine profile in adult-onset Still’s disease: Comparison with systemic juvenile idiopathic arthritis. Clin Immunol 2016; 169: 8-13.
[http://dx.doi.org/10.1016/j.clim.2016.05.010] [PMID: 27263804]
[9]
Nirmala N, Brachat A, Feist E, et al. Gene-expression analysis of adult-onset Still’s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity. Pediatr Rheumatol Online J 2015; 13(1): 50.
[http://dx.doi.org/10.1186/s12969-015-0047-3] [PMID: 26589963]
[10]
Pay S, Türkçapar N, Kalyoncu M, et al. A multicenter study of patients with adult-onset Still’s disease compared with systemic juvenile idiopathic arthritis. Clin Rheumatol 2006; 25(5): 639-44.
[http://dx.doi.org/10.1007/s10067-005-0138-5] [PMID: 16365690]
[11]
Luthi F, Zufferey P, Hofer MF, So AK. “Adolescent-onset Still’s disease”: Characteristics and outcome in comparison with adult-onset Still’s disease. Clin Exp Rheumatol 2002; 20(3): 427-30.
[PMID: 12102485]
[12]
Lin SJ, Chao HC, Yan DC. Different articular outcomes of Still’s disease in Chinese children and adults. Clin Rheumatol 2000; 19(2): 127-30.
[http://dx.doi.org/10.1007/s100670050030] [PMID: 10791624]
[13]
Tanaka S, Matsumoto Y, Ohnishi H, et al. Comparison of clinical features of childhood and adult onset Still’s disease. Ryumachi 1991; 31(5): 511-8.
[PMID: 1767345]
[14]
Kudela H, Drynda S, Lux A, Horneff G, Kekow J. 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; 3(1): 4.
[http://dx.doi.org/10.1186/s41927-019-0053-z] [PMID: 30886992]
[15]
Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992; 19(3): 424-30.
[PMID: 1578458]
[16]
Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 2006; 65(5): 564-72.
[http://dx.doi.org/10.1136/ard.2005.042143] [PMID: 16219707]
[17]
Gurion R, Lehman TJA, Moorthy LN. Systemic arthritis in children: A review of clinical presentation and treatment. Int J Inflamm 2012; 2012: 1-16.
[http://dx.doi.org/10.1155/2012/271569] [PMID: 22235382]
[18]
Ruscitti P, Rago C, Breda L, et al. Macrophage activation syndrome in Still’s disease: Analysis of clinical characteristics and survival in paediatric and adult patients. Clin Rheumatol 2017; 36(12): 2839-45.
[http://dx.doi.org/10.1007/s10067-017-3830-3] [PMID: 28914368]
[19]
Maria ATJ, Le Quellec A, Jorgensen C, Touitou I, Rivière S, Guilpain P. Adult onset Still’s disease (AOSD) in the era of biologic therapies: Dichotomous view for cytokine and clinical expressions. Autoimmun Rev 2014; 13(11): 1149-59.
[http://dx.doi.org/10.1016/j.autrev.2014.08.032] [PMID: 25183244]
[20]
Ichida H, Kawaguchi Y, Sugiura T, et al. Clinical manifestations of adult-onset Still’s disease presenting with erosive arthritis: Association with low levels of ferritin and interleukin-18. Arthritis Care Res (Hoboken) 2014; 66(4): 642-6.
[http://dx.doi.org/10.1002/acr.22194] [PMID: 24124073]
[21]
Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: Diagnosis, genetics, pathophysiology and treatment. Genes Immun 2012; 13(4): 289-98.
[http://dx.doi.org/10.1038/gene.2012.3] [PMID: 22418018]
[22]
Kelly A, Ramanan AV. Recognition and management of macrophage activation syndrome in juvenile arthritis. Curr Opin Rheumatol 2007; 19(5): 477-81.
[http://dx.doi.org/10.1097/BOR.0b013e32825a6a79] [PMID: 17762614]
[23]
Delplanque M, Pouchot J, Ducharme-Bénard S, et al. AA amyloidosis secondary to adult onset Still’s disease: About 19 cases. Semin Arthritis Rheum 2020; 50(1): 156-65.
[http://dx.doi.org/10.1016/j.semarthrit.2019.08.005] [PMID: 31488308]
[24]
Nigrovic PA, Mannion M, Prince FHM, Zeft A, Rabinovich CE, van Rossum MAJ. 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(2): 545-55.
[25]
Sugiura T, Kawaguchi Y, Harigai M, et al. Association between adult-onset Still’s disease and interleukin-18 gene polymorphisms. Genes Immun 2002; 3(7): 394-9.
[http://dx.doi.org/10.1038/sj.gene.6363922] [PMID: 12424620]
[26]
Chen DY, Lan JL, Lin FJ, Hsieh TY. Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still’s disease. J Rheumatol 2004; 31(11): 2189-98.
[PMID: 15517632]
[27]
Nigrovic PA. Review: Is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis? Arthritis Rheumatol 2014; 66(6): 1405-13.
[http://dx.doi.org/10.1002/art.38615] [PMID: 24623686]
[28]
Chen DY, Chen YM, Lan JL, Lin CC, Chen HH, Hsieh CW. Potential role of Th17 cells in the pathogenesis of adult-onset Still’s disease. Rheumatology (Oxford) 2010; 49(12): 2305-12.
[http://dx.doi.org/10.1093/rheumatology/keq284] [PMID: 20837500]
[29]
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): 2385-95.
[http://dx.doi.org/10.1056/NEJMoa1112802] [PMID: 23252525]
[30]
Ravelli A, Consolaro A, Horneff G, et al. Treating juvenile idiopathic arthritis to target: Recommendations of an international task force. Ann Rheum Dis 2018; 77(6) annrheumdis-2018-213030
[http://dx.doi.org/10.1136/annrheumdis-2018-213030] [PMID: 29643108]
[31]
Colafrancesco S, Manara M, Bortoluzzi A, Serban T, Bianchi G, Cantarini L. Thu0569 management of adult-onset Still’s disease (aosd) with Il-1 inhibitors: Evidence and consensus based statements by a panel of Italian experts. Ann Rheum Dis 2019; 78(2): 575-6.
[32]
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(1): 256.
[http://dx.doi.org/10.1186/s13075-017-1462-2] [PMID: 29166924]
[33]
Guzman J, Kerr T, Ward LM, et al. Growth and weight gain in children with juvenile idiopathic arthritis: Results from the ReACCh-Out cohort. Pediatr Rheumatol Online J 2017; 15(1): 68.
[http://dx.doi.org/10.1186/s12969-017-0196-7] [PMID: 28830457]
[34]
Sota J, Rigante D, Ruscitti P, et al. Anakinra drug retention rate and predictive factors of long-term response in systemic Juvenile idiopathic arthritis and adult onset Still disease. Front Pharmacol 2019; 10: 918.
[http://dx.doi.org/10.3389/fphar.2019.00918] [PMID: 31507416]
[35]
Vercruysse F, Barnetche T, Lazaro E, et al. Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy. Arthritis Res Ther 2019; 21(1): 53.
[http://dx.doi.org/10.1186/s13075-019-1838-6] [PMID: 30755262]
[36]
Colina M, Zucchini W, Ciancio G, Orzincolo C, Trotta F, Govoni M. The evolution of adult-onset Still disease: An observational and comparative study in a cohort of 76 Italian patients. Semin Arthritis Rheum 2011; 41(2): 279-85.
[http://dx.doi.org/10.1016/j.semarthrit.2010.12.006] [PMID: 21377714]
[37]
Mitrovic S, Fautrel B. Clinical phenotypes of adult-onset Still’s disease: New insights from pathophysiology and literature findings. J Clin Med 2021; 10(12): 2633.
[http://dx.doi.org/10.3390/jcm10122633] [PMID: 34203779]
[38]
Li R, Liu X, Chen G, et al. Clinical phenotypes and prognostic factors of adult-onset Still’s disease: Data from a large inpatient cohort. Arthritis Res Ther 2021; 23(1): 300.
[http://dx.doi.org/10.1186/s13075-021-02688-4] [PMID: 34879864]
[39]
Yin R, Wang G, Yang X, et al. Identification of prognostic factors and construction of a nomogram for patients with relapse/refractory adult-onset Still’s disease. Clin Rheumatol 2021; 40(10): 3951-60.
[http://dx.doi.org/10.1007/s10067-021-05722-7] [PMID: 34002352]
[40]
Prieur AM, Chèdeville G. Prognostic factors in juvenile idiopathic arthritis. Curr Rheumatol Rep 2001; 3(5): 371-8.
[http://dx.doi.org/10.1007/s11926-996-0006-6] [PMID: 11564367]

Rights & Permissions Print Cite
Article Metrics
7
© 2024 Bentham Science Publishers | Privacy Policy