Schnitzler syndrome: response to anakinra in two cases and a review of the literature
| Autor: | Birger Kränke, Gunter J. Sturm, Elisabeth Aberer, Christian Schuster, Emad Arbab, Werner Aberer |
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| Rok vydání: | 2009 |
| Předmět: |
Adult
Male musculoskeletal diseases medicine.medical_specialty Dermatology Immunopathology Humans Medicine In patient Schnitzler Syndrome Anakinra business.industry Remission Induction Complete remission Middle Aged medicine.disease Pathophysiology Surgery Interleukin 1 Receptor Antagonist Protein Monoclonal gammopathy Treatment Outcome Schnitzler syndrome Antirheumatic Agents Female medicine.symptom business Rare disease medicine.drug |
| Zdroj: | International Journal of Dermatology. 48:1190-1194 |
| ISSN: | 1365-4632 0011-9059 |
| DOI: | 10.1111/j.1365-4632.2009.04151.x |
| Popis: | Background Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation. The pathophysiology is still unknown, although various autoantibody-mediated mechanisms have been described. Complete remission of symptoms has been reported recently in patients with Schnitzler syndrome treated with anakinra, an interleukin-1 receptor antagonist. Methods Two patients with Schnitzler syndrome treated with anakinra therapy are presented. Results We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy. A review of the published literature on the treatment of Schnitzler syndrome with anakinra is presented. Conclusions Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms. |
| Databáze: | OpenAIRE |
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