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Background: Adult-onset Still’s disease (AOSD) is a rare multisystemic inflammatory disorder, and is diagnosed by exclusion. AOSD is generally treated with nonsteroidal antiinflammatory drugs, corticosteroids, and conventional disease modifiying antirheumatic drugs (cDMARDs). Biological disease modifiying antirheumatic drug (bDMARD) therapy are recommended in AOSD patients who are refractory to tradional therapy, and bDMARDs is becoming increasingly important in AOSD treatment.
Objectives: To evaluate the use of bDMARDs and drug survival in AOSD patients.
Methods: TURKBIO registry is the Turkish version of Danish DANBIO rheumatological database which has been established in 2011. All patients with AOSD who received biological agents registered in TURKBIO registry between dates of October 2011 and October 2019 were included in this study. The demographic data, response of therapy, frequency of using and switching biological agents were collected.
Results: As of October, 21 AOSD patients were recruited. Mean age of patients was 34.6±7.3 (min-max: 24-49) years, mean disease duration was 9.3±7.4 (min-max: 1-22) years, and 57.1% of patients was female. Mean duration from onset to start of bDMARDs was 7±6.1 (min-max: 0.5-21) years. It was observed that 13 patients (61.9%) received tocilizumab (TCZ), 6 patients (28.6%) received IL-1 inhibitors (5 anakinra and one canakinumab), 2 patients (9.5%) received certolizumab and one patient (4.8%) received etanercept as a first-line bDMARDs. The most frequently used biological agents in current treatment were as follows: 52.4% of patients received TCZ and 33.3% received IL-1 inhibitors (4 anakinra, 3 canakinumab), and the most frequently used concomitant drugs were methotrexate (47.6%) and hydroxychloroquine (14.3%). The switching rate was 33.3%, and in half of them the reason of switching was adverse events. The median drug survival for bDMARDs was 28.6 months (
Demographic, laboratory features and management of AOSD
| (median;25-75) | n=21 |
|---|---|
| Age (year) | 34.7 (28.3-40.6) |
| Gender (Female) n(%) | 12 (57.1) |
| Disease duration (year) | 8 (2-17) |
| Duration from onset to start of bDMARs (year) | 6 (1.5-10) |
| ESR (on onset) | 44 (21-66) |
| CRP (on onset) | 65 (3.1-108) |
| Current bDMARDs n(%) | |
| Tocilizumab | 11(52.4) |
| IL-1 inhibitors | 7 (33.3) |
| Etanercept | 1 (4.8) |
| Certolizumab | 2 (9.5) |
| Concomitant cDMARD n(%) | |
| Methotrexate | 10 (47.6) |
| Leflunomide | 4 (19) |
| Sulfasalazine | 1 (4.8) |
| Hydroxychloroquine | 3 (14.3) |
| bDMARDs Survival (months) | 28.6 (5.5-75) |
| Switching Rate n(%) | 7 (33.3) |
| Adverse Event n(%) | 3 (14.3) |
Conclusion: This is the first evaluation of AOSD patients who used biological agents from TURKBIO registry. According our data, TCZ and anti-IL1 agents were the most frequent biological choices. The limitation of this study was the low number of the patients with AOSD who used biological agents.
REFERENCES:
[1].Zhou S, Qiao J, Bai J, Wu Y, Fang H. Biological therapy of traditional therapy-resistant adult-onset Still’s disease: an evidence-based review. Ther Clin Risk Manag 2018;14:167-71.
Acknowledgments: None
Disclosure of Interests: None declared