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. 2021 Feb 2;11:635823. doi: 10.3389/fphar.2020.635823

TABLE 2.

Predictors for biologic treatment response in SJIA.

Ref Medication Study design N patients Results
Gattorno et al. (2008) ANA Prospective 22 (10 responders) Complete responders had a lower number of active joints vs. non responders (median 3.5 vs. 7) and a higher number of neutrophils (median 19.3 vs. 9.1 × 103/mm3),
Nigrovic et al. (2011) ANA Retrospective, multi-centre 46 Incomplete responders were younger at onset vs. complete responders (median age 5.2 vs. 10.2 years), (OR 1.5 per year; 95% CI 1.1–2.0)
Holzinger et al. (2012) ANA, ETN Prospective 52 12 on biologics •MRP8/14 decreased markedly in responders to biologic treatment (12/12) and in responders (6/12) to methotrexate
•MRP8/14 detects flares vs. inactive disease with outstanding diagnostic accuracy (AUC: 0.957 ± 0.019)
•MRP8/14 > 740 ng/ml can predict relapse in next 6 months (AUC: 0.91), 13/26 inactive patients relapsed
Vastert et al. (2014) ANA Prospective single-centre 20 (15 responders) •S100A8/9 (MRP8/14), S100A12 and IL-8 decreased markedly in responders (ACR Pedi 90) at 3 months
•Lower levels of IL-8, S100A12, S100A8/9 at 3 months in 7/15 patients with ID who succeeded to discontinue treatment within a year (significant only for S100A12)
Pardeo et al. (2015) ANA Retrospective, single-centre 25 (14 responders) Earlier treatment from disease onset associated with ID at 6 months (median 1.9 vs. 24.5 months).
Saccomanno et. al. (2019) ANA Retrospective single-centre 62 (24 responders) Predictors of complete clinical response at 1 year included:
•Disease duration ≤3.9 years (OR 6.78; 95% CI 1.30–35.27),
•Active joint count ≤10 (OR 8.25; 95% CI 1.26–53.91),
•Ferritin >444 ng/ml (OR 4.75; 95% CI 1.16–19.50),
•Systemic manifestation score >3 (OR 6.44; 95% CI 1.38–24.62), AUC: 0.83
Kearsley-Fleet et al. (2019) ANA, TCZ Prospective, multi-centre 76 Baseline characteristics not associated with response (ACR Pedi 90, MDA or ID)
Ter Haar et al. (2019) ANA Prospective, single-centre 42 (32 ID at 1 year) •ID at 1 month after ANA treatment predicted ID at 1 year (OR 27; 95% CI 4.17–539.74), AUC: 0.84
•Neutrophils>9 × 109/L at baseline predict ID at 1 year (OR 38.67; 95% CI 6.53–362.73), AUC: 0.85
Ruperto et al. (2018) CAN Open-label, long-term extension study 144 (96 early responders) Early responders (completed glucocorticoid tapering in part I of trial 2) achieved greater decrease in JADAS during the study as compared with late responders (mixed model; p < 0.01)
Bielak et al. (2018) TCZ Prospective, multi-centre 46 •7/17 (41%) patients showing inactive disease at the last visit had a response to TCZ within 5 weeks
•Polycyclic course was associated with greater odds of clinical response (OR 7.0; 95% CI 1.8–27.2) compared to monocyclic or polyarticular course of SJIA

ANA, Anakinra; ACR, American college of Rheumatology; AUC, area under the curve; CAN, canakinumab; CI, confidence interval; ETN, etanercept; ID, clinically inactive disease; MDA, minimal disease activity; MRP, myeloid-related protein; OR, odds ratio; Pedi, pediatric; SJIA, systemic juvenile idiopathic arthritis; TCZ, tocilizumab.