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Efficacy and safety of the interleukin‐1 antagonist rilonacept in S chnitzler syndrome: an open‐label study
Author(s) -
Krause K.,
Weller K.,
Stefaniak R.,
Wittkowski H.,
Altrichter S.,
Siebenhaar F.,
Zuberbier T.,
Maurer M.
Publication year - 2012
Publication title -
allergy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.363
H-Index - 173
eISSN - 1398-9995
pISSN - 0105-4538
DOI - 10.1111/j.1398-9995.2012.02843.x
Subject(s) - medicine , anakinra , canakinumab , rash , adverse effect , gastroenterology , disease
Background S chnitzler 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 S chnitzler 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 ( NCT 01045772 [ ClinicalTrials.gov Identifier]; Eudra CT #2006‐004290‐97).