Session Title: Pediatric Rheumatology – Clinical Poster I: JIA (0241–0265)
Session Type: Poster Session A
Session Time: 8:30AM-10:30AM
Background/Purpose: Systemic-onset juvenile idiopathic arthritis (sJIA) is a pediatric autoinflammatory condition, known for significant variability between patients in its severity and long-term outcomes. The classification of disease course into monophasic, polyphasic (intermittent) and persistent disease has been commonly used, with polyphasic disease usually recognized in a small portion of patients1,2 . However, this proportion was established according to data mostly collected before the biologic IL-1 and IL-6 inhibitors were available. It has been recently suggested 3,4 that these medications, now the mainstay of treatment in sJIA, can potentially alter the long-term course of the disease, especially with earlier use.
Methods: A multi-center, retrospective chart review was conducted from 3 hospitals in Israel and 2 in the US, involving patients diagnosed with sJIA between 1998-2019, with a minimum follow-up of 1 year. Disease course classification was done according to previously released definitions.1 Remission was defined as inactive disease (no active signs or symptoms and no elevation of inflammatory markers) while not receiving any medications for a period of at least 3 months. Polyphasic disease was defined as at least one disease relapse after a period of drug-free remission. Persistent disease was defined as at least 2 years of disease (either active or in remission on immunosuppressive medication) with no drug-free remissions. Monophasic disease was defined as a single episode lasting less than 2 years.
Results: : 85 patients met the inclusion criteria (mean follow up 3.6 years, SD ± 2.8). 54 of the patients (63.5%) were female; median age at diagnosis was 5.8 years (IQR = 5.9). 67 (78.9%) were diagnosed in 2012 or later, when IL-1 and IL-6 inhibitors became widely used. 56 (65.9%) were treated with a biologic drug during their disease course. The rates of monophasic, polyphasic and persistent disease were 43.5%, 43.5% and 12.9%, respectively, with a higher-than-expected rate of polyphasic disease and a lower rate of persistent disease than previously published. 1,2,5
Conclusion: In the age of IL-1 and IL-6 inhibitors, polyphasic sJIA disease course may be more common than previously described, suggesting that cytokine blockers may potentially alter the natural history of this disease.
1 Singh-Grewal et al, Arthritis Rheum. 20062 Barut et al, Int J Rheum Dis. 2019
3 Henderson LA et al, JCI Insight. 2020 4Kessel C et al. (2020) Arthritis Rheumatol
5 Lomater et al, J Rheumatol. 2000
To cite this abstract in AMA style:Marmor I, Semo Oz R, Hendel A, Hazan G, Baszis K, French A, Edens C, Tirosh i, Butbul Aviel Y, Harel L, Amarilyo G. Proportion of Patients with a Polyphasic Disease Course in Systemic-onset Juvenile Idiopathic Arthritis May Be Higher in the Age of Cytokine Inhibitors [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/proportion-of-patients-with-a-polyphasic-disease-course-in-systemic-onset-juvenile-idiopathic-arthritis-may-be-higher-in-the-age-of-cytokine-inhibitors/. Accessed January 30, 2023.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/proportion-of-patients-with-a-polyphasic-disease-course-in-systemic-onset-juvenile-idiopathic-arthritis-may-be-higher-in-the-age-of-cytokine-inhibitors/