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- Title
Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study.
- Authors
Krause, K.; Weller, K.; Stefaniak, R.; Wittkowski, H.; Altrichter, S.; Siebenhaar, F.; Zuberbier, T.; Maurer, M.
- Abstract
Background Schnitzler syndrome (SchS) is a rare disease with suspected autoinflammatory background that shares several clinical symptoms, including urticarial rash, fever episodes, arthralgia, and bone and muscle pain with cryopyrin-associated periodic syndromes ( CAPS). Cryopyrin-associated periodic syndromes respond to treatment with interleukin-1 antagonists, and single case reports of Schnitzler syndrome have shown improvement following treatment with the interleukin-1 blocker anakinra. This study evaluated the effects of the interleukin-1 antagonist rilonacept on the clinical signs and symptoms of SchS. Methods Eight patients with SchS were included in this prospective, single-center, open-label study. After a 3-week baseline, patients received a subcutaneous loading dose of rilonacept 320 mg followed by weekly subcutaneous doses of 160 mg for up to 1 year. Efficacy was determined by patient-based daily health assessment forms, physician's global assessment (PGA), and measurement of inflammatory markers including C-reactive protein ( CRP), serum amyloid A ( SAA), and S100 calcium-binding protein A12 (S100A12). Results Treatment with rilonacept resulted in a rapid clinical response as demonstrated by significant reductions in daily health assessment scores and PGA scores compared with baseline levels ( P < 0.05). These effects, which were accompanied by reductions in CRP and SAA, continued over the treatment duration. Rilonacept treatment was well tolerated. There were no treatment-related severe adverse events and no clinically significant changes in laboratory safety parameters. Conclusion Rilonacept was effective and well tolerated in patients with SchS and may represent a promising potential therapeutic option ( NCT01045772 [ Identifier]; Eudra CT #2006-004290-97).
- Subjects
INTERLEUKIN-1; PHARMACODYNAMICS; SCHNITZLER syndrome; AUTOIMMUNE diseases; CLINICAL trials; C-reactive protein; PHYSICIANS; PATIENTS; THERAPEUTICS
- Publication
Allergy, 2012, Vol 67, Issue 7, p943
- ISSN
0105-4538
- Publication type
Article
- DOI
10.1111/j.1398-9995.2012.02843.x