Autoinflammatory & rare systemic diseases
IL-1 targeting agents in Schnitzler syndrome: a multicentre, real-world study from the international AutoInflammatory Disease Alliance (AIDA) Network Schnitzler Registry
Authors' conclusion
The present study confirms the high effectiveness of IL-1 inhibitors in controlling SchS, including the complete and partial response rates and the long-term survival.
Study context
- Population
- Patients with Schnitzler syndrome (SchS) treated with IL-1 inhibitors, international AIDA Registry37 treatment lines
- Participants
- 28 patients
- Exposure
- Anakinra vs canakinumab
- Outcome
- Long-term effectiveness (complete and partial response) and safety of IL-1 inhibitors
Methodological detailsmulticentre, real-world, registry-based (international AIDA Network Schnitzler Registry)
- Multicentre
- Real-world
- Registry-based (international AIDA Network Schnitzler Registry)
Main finding
As reported in the abstract
Complete and partial response
Complete response
Partial response
Observational data: associations, not effects.
Further findings
Lymphadenopathy as independent predictor of discontinuation due to loss of effectiveness
| HR (95% CI) | P value | ||
|---|---|---|---|
| Lymphadenopathy | 7.78 (1.27–47.9) | P = 0.027 | |
| 0.050.21520Hazard ratio (log scale) |
Overall anakinra and canakinumab drug retention · 12, 36 and 60 months
- Drug retention at 12 months
- 85.6%
- Drug retention at 36 months
- 81.7%
- Drug retention at 60 months
- 64.7%
Probability of discontinuing IL-1 inhibitors due to loss of effectiveness · 12, 36 and 60 months
- At 12 months
- 9.6%
- At 36 months
- 13.7%
- At 60 months
- 24.5%
Maximum IgG M-protein levels, partial vs complete response
- Significantly higher in partial than complete responders
- P = 0.032
Original publication
IL-1 targeting agents in Schnitzler syndrome: a multicentre, real-world study from the international AIDA Network Schnitzler Registry. Clin Exp Rheumatol. Published 23 October 2025. doi:10.
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