Optimized Treatment of Interleukin (IL-1)-Mediated Autoinflammatory Diseases: Impact of Disease Activity-Based Treatment Adjustments
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patient Related Data
2.2. Treatment Regimen and Definitions
2.3. Definition of Disease Activity
2.4. Outcome
2.5. Analysis
3. Results
3.1. Disease Activity over Time
3.2. Treatment Adjustments
3.2.1. Disease Activity-Based Treatment Adjustments
3.2.2. Specific Dosing Regimen
3.3. Impact on Disease Activity Trajectories
3.3.1. PGA and PPGA
3.3.2. Parameter Trajectories/Changes
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Total n = 56 (100%) | FMF n = 46 (82%) | CAPS n = 9 (16%) | TRAPS n = 1 (2%) | |
---|---|---|---|---|
Demographic characteristics | ||||
Female 1 | 19 (34) | 17 (37) | 2 (22) | 0 |
Symptom onset, age in years 2 | 2.5 (0.5; 4.1) | 2.9 (1.6; 4.7) | 0.3 (0.2; 0.5) | 2.7 |
Diagnosis, age in years 2 | 4.9 (3.0; 7.7) | 5.1 (3.6; 7.5) | 2.8 (1.9; 4.5) | 12.9 |
First study visit, age in years 2 | 4.9 (3.3; 8.1) | 5.1 (3.7; 8.0) | 3.2 (1.9; 4.5) | 13.0 |
Follow-up, years 2 | 2.1 (1.4; 2.7) | 1.9 (1.4; 2.5) | 2.5 (2.3; 2.9) | 3.0 |
Genetic variants | ||||
Pathogenic/likely pathogenic 1 | 28 (50) | 25 (54) | 2 (22) | 1 (100) |
VUS 1 | 11 (20) | 4 (9) | 7 (78) | 0 |
Total n = 56 | FMF n = 46 | CAPS n = 9 | TRAPS n = 1 | |
---|---|---|---|---|
First episode of increased disease activity, n (%) | 36 (64) | 30 (65) | 5 (56) | 1 (100) |
Treatment adjustment in 28 out of 36 children * | ||||
New treatment start, n (%) | 19 (34) | 15 (33) | 3 (33) | 1(100) |
Treatment switch n, (%) | 4 (7) | 3 (7) | 1 (11) | 0 (0) |
Dose increase n, (%) | 8 (14) | 7 (15) | 1 (11) | 0 (0) |
Administration frequency increase, n (%) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
Subsequent episode of increased disease activity, n (%) | 17 (30) | 13 (28) | 4 (44) | 0 (0) |
Treatment adjustments in 7 out of 17 children * | ||||
New treatment start, n (%) | 2 (4) | 2 (4) | 0 (0) | 0 (0) |
Treatment switch, n (%) | 1 (2) | 1 (2) | 0 (0) | 0 (0) |
Dose increase, n (%) | 3 (5) | 1 (2) | 2 (22) | 0 (0) |
Administration frequency increase, n (%) | 1 (2) | 0 (0) | 1 (11) | 0 (0) |
IL-1 AID Subgroups | |||||||||
CAPS | FMF | TRAPS | |||||||
beta 1 | 95%CI | p Value | beta 1 | 95%CI | p Value | beta 1 | 95%CI | p Value | |
PGA | −0.12 | −0.16; −0.08 | <0.001 | −0.15 | −0.24; −0.06 | 0.001 | −0.11 | −0.17; −0.06 | <0.001 |
PPGA | −0.09 | −0.19; 0.02 | 0.100 | −0.11 | −0.32; 0.10 | 0.304 | −0.08 | −0.21; 0.05 | 0.247 |
CRP | −0.05 | −0.10; −0.01 | 0.026 | −0.10 | −0.23; 0.03 | 0.130 | −0.04 | −0.09; 0.01 | 0.093 |
SAA | −2.49 | −5.68; 0.71 | 0.127 | −4.21 | −10.62; 2.21 | 0.199 | −2.26 | −5.99; 1.47 | 0.236 |
Genotypes | |||||||||
Pathogenic/likely pathogenic variants | VUS | No genetic testing/no VUS or (likely) pathogenic variants detected | |||||||
beta 1 | 95%CI | p value | beta 1 | 95%CI | p value | beta 1 | 95%CI | p value | |
PGA | −0.13 | −0.19; −0.08 | <0.001 | −0.09 | −0.17; −0.01 | 0.020 | −0.15 | −0.26; −0.05 | 0.004 |
PPGA | −0.09 | −0.23; 0.06 | 0.225 | −0.08 | −0.22; 0.06 | 0.280 | −0.07 | −0.28; 0.14 | 0.526 |
CRP | −0.07 | −0.16; 0.01 | 0.073 | −0.05 | −0.12; 0.01 | 0.111 | −0.04 | −0.13; 0.05 | 0.444 |
SAA | −3.18 | −8.97; 2.62 | 0.283 | −2.67 | −6.15; 0.80 | 0.132 | −1.73 | −10.25; 6.79 | 0.691 |
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Welzel, T.; Zapf, B.; Klotsche, J.; Satirer, Ö.; Benseler, S.M.; Kuemmerle-Deschner, J.B. Optimized Treatment of Interleukin (IL-1)-Mediated Autoinflammatory Diseases: Impact of Disease Activity-Based Treatment Adjustments. J. Clin. Med. 2024, 13, 2319. https://doi.org/10.3390/jcm13082319
Welzel T, Zapf B, Klotsche J, Satirer Ö, Benseler SM, Kuemmerle-Deschner JB. Optimized Treatment of Interleukin (IL-1)-Mediated Autoinflammatory Diseases: Impact of Disease Activity-Based Treatment Adjustments. Journal of Clinical Medicine. 2024; 13(8):2319. https://doi.org/10.3390/jcm13082319
Chicago/Turabian StyleWelzel, Tatjana, Beate Zapf, Jens Klotsche, Özlem Satirer, Susanne M. Benseler, and Jasmin B. Kuemmerle-Deschner. 2024. "Optimized Treatment of Interleukin (IL-1)-Mediated Autoinflammatory Diseases: Impact of Disease Activity-Based Treatment Adjustments" Journal of Clinical Medicine 13, no. 8: 2319. https://doi.org/10.3390/jcm13082319