ArticleArthritis research & therapy2015
The role of interleukin-1 beta in the pathophysiology of Schnitzler's syndrome.
Article in Arthritis research & therapy, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 52 citations in OpenAlex.
- Long-term anakinra therapy in Schnitzler syndrome: real-world evidence on remission of neutrophilic dermatitis and systemic inflammation, safety, biomarker insights and dose optimization.Frontiers in immunology · 2026Observational
- Schnitzler syndrome.Dermatologie (Heidelberg, Germany) · 2025Review
- Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome.International journal of molecular sciences · 2025Review
- The effect of immunomodulatory therapy with recombinant human interferon alpha-2β on blood cytokine levels in children with recurrent episodes of acute obstructive bronchitis.Clinical and experimental immunology · 2023Article
- Therapy resistant urticaria as a long-term symptom of an incomplete Schnitzler syndrome.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2023Article
- Schnitzler syndrome.BMJ case reports · 2023Article
- Canakinumab leads to rapid reduction of neutrophilic inflammation and long-lasting response in Schnitzler syndrome.Frontiers in medicine · 2023Article
- The Expanding Spectrum of Autoinflammatory Diseases.Internal medicine (Tokyo, Japan) · 2023Article
- Cutaneous manifestations of monoclonal gammopathy.Blood cancer journal · 2022Review
- New insights on multigenic autoinflammatory diseases.Therapeutic advances in musculoskeletal disease · 2022Review
- Efficacy of interleukin-1 blockade in Schnitzler's syndrome without detectable monoclonal gammopathy: a case-based review.Clinical rheumatology · 2021Review
- Hereditary systemic autoinflammatory diseases and Schnitzler's syndrome.Rheumatology (Oxford, England) · 2019Review
- The IL-1 family of cytokines and receptors in rheumatic diseases.Nature reviews. Rheumatology · 2019Review
- Cell death-mediated cytokine release and its therapeutic implications.The Journal of experimental medicine · 2019Review
- Refractory urticaria and the importance of diagnosing Schnitzler's syndrome.BMJ case reports · 2019Article
- [Schnitzler syndrome].Zeitschrift fur Rheumatologie · 2019Article
- Inhibition of interleukin-1 receptor-associated kinase 1 (IRAK1) as a therapeutic strategy.Oncotarget · 2018Review
- [Schnitzler syndrome].Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete · 2018Article
- A Review of Recent Advances Using Tocilizumab in the Treatment of Rheumatic Diseases.Rheumatology and therapy · 2018Review
- Increasing the Interval of Canakinumab Administration Effectively Supports the Remission of Schnitzler's Syndrome.Case reports in rheumatology · 2018Article
Corrections and comments
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Authors and funding
12 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionSchnitzler's syndrome (SchS) is a disabling autoinflammatory disorder, characterized by a chronic urticarial rash, an M-protein, arthralgia, and other signs of systemic inflammation. Anti-interleukin-1 (IL-1) beta antibodies are highly effective, but the pathophysiology is still largely unknown. Here we studied the effect of in-vivo IL-1 inhibition on serum markers of inflammation and cellular immune responses.
methodsEight patients with SchS received monthly subcutaneous (s.c.) injections with 150 mg canakinumab for six months. Blood was drawn for measurement of serum markers of inflammation (12 times per patient) and for functional and phenotypic analysis of both freshly isolated and toll-like receptor (TLR)-ligand-stimulated peripheral blood mononuclear cells (PBMCs) (five times per patient). All data were compared to results of healthy controls.
resultsIL-6 levels in serum and in lysates of freshly isolated PBMCs and serum myeloid-related protein (MRP8)/14 and S100A12 levels correlated with disease activity. In vitro, LPS stimulation resulted in higher IL-6 and IL-1 beta production in PBMCs from symptomatic SchS patients compared to healthy controls, whereas patient cells were relatively hyporesponsive to poly:IC and Pam3Cys. The mRNA microarray of PBMCs showed distinct transcriptomes for controls, symptomatic patients and anti-IL-1-treated patients. Numbers of T- and B-cell subsets as well as M-protein concentrations were not affected by IL-1 inhibition. Free light chain levels were elevated in 4 out of 8 patients.
conclusionsIn conclusion, patient PBMCs are hyperresponsive to LPS, and clinical efficacy of IL-1 beta inhibition in patients with SchS is associated with in-vivo and ex-vivo suppression of inflammation. Interestingly, patient PBMCs showed divergent responses to TLR2/6, TLR3 and TLR4 ligands. Our data underscore that IL-1 beta plays a pivotal role in SchS.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.