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A rare, underdiagnosed disorder in adults characterized by recurrent febrile rash, bone and/or joint pain, enlarged lymph nodes, fatigue, a monoclonal IgM component, leukocytosis and systemic inflammatory response.
No approved treatments are currently available for Schnitzler syndrome. An additional 1 compound holds orphan drug designation.
While no drugs are FDA-approved specifically for Schnitzler syndrome, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for Schnitzler syndrome. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
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Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
2 clinical trials registered, 1 recruiting. Interventions under study include other interventions. Research is sponsored by a mix of industry and academic institutions.
47 publications have been identified in PubMed for Schnitzler syndrome. Research spans Case Report / Case Series (40%), Review / Meta-Analysis (36%), and Other (6%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 19 | 40% |
Data assembled from 5 of 12 sources · Last updated Sep 17, 2026, 7:19 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about Schnitzler syndrome
Designated
Exclusivity End |
|---|
Designation Status |
|---|
heterodimeric fusion protein that binds to IL-1 beta consisting of human extracellular domain of IL1 receptor and a mutant Fc fragment of human IgG1 and part of human IL1 receptor accessory protein | heterodimeric fusion protein that binds to IL-1 beta consisting of human extracellular domain of IL1 receptor and a mutant Fc fragment of human IgG1 and part of human IL1 receptor accessory protein | R-Pharm Overseas | 2018 | — | Designated |
2 trials found
Research summaries |
17 |
36% |
Other research | 3 | 6% |
Laboratory research | 3 | 6% |
Disease patterns and progression | 3 | 6% |
Clinical study results | 2 | 4% |
Sikora M (2026). [PMID: 42158864](https://pubmed.ncbi.nlm.nih.gov/42158864/). *Front Immunol*. [Epidemiology / Natural History]
Zhou Y (2026). [PMID: 41944016](https://pubmed.ncbi.nlm.nih.gov/41944016/). *Allergy*. [Other]
Mir TH (2026). [PMID: 41740896](https://pubmed.ncbi.nlm.nih.gov/41740896/). *J Am Acad Dermatol*. [Other]
Calabrese L (2026). [PMID: 42099270](https://pubmed.ncbi.nlm.nih.gov/42099270/). *Clin Exp Rheumatol*. [Other]
Roskoski R Jr (2026). [PMID: 41937093](https://pubmed.ncbi.nlm.nih.gov/41937093/). *Pharmacol Res*. [Review / Meta-Analysis]
Velusamy B (2026). [PMID: 41542300](https://pubmed.ncbi.nlm.nih.gov/41542300/). *JAAD case reports*. [Case Report / Case Series]
Santaniello U (2026). [PMID: 41838481](https://pubmed.ncbi.nlm.nih.gov/41838481/). *Italian journal of dermatology and venereology*. [Case Report / Case Series]
De Santos Belinchón S (2026). [PMID: 41635972](https://pubmed.ncbi.nlm.nih.gov/41635972/). *Internal medicine journal*. [Review / Meta-Analysis]
Sra MS (2026). [PMID: 42215470](https://pubmed.ncbi.nlm.nih.gov/42215470/). *Blood Cancer J*. [Review / Meta-Analysis]
Hiroyasu S (2026). [PMID: 41243422](https://pubmed.ncbi.nlm.nih.gov/41243422/). *The Journal of dermatology*. [Review / Meta-Analysis]
AI-curated news mentioning Schnitzler syndrome
Updated May 7, 2026
A multicenter study from the AIDA Network Schnitzler Registry evaluates IL-1 targeting agents in patients with Schnitzler syndrome. The findings contribute to understanding treatment efficacy in this rare disease.