Schnitzler syndrome: treatment failure to rituximab but response to anakinra.

Abstract

Schnitzler syndrome is a rare disease characterized by a chronic urticarial rash and a monoclonal gammopathy. The exact pathogenesis is still uncertain and treatment remains a challenge. Here, we report a patient who was only recently given the diagnosis of Schnitzler syndrome although the typical symptoms had been present for about 6 years. Administration of the B-cell antibody rituximab did not exert any beneficial effect despite effective elimination of B cells and a reduction of the paraprotein. In contrast, injection of the interleukin-1-receptor antagonist anakinra caused a complete remission within a few days.

• Authors: Elisabeth Eiling, Maike Möller, Inga Kreiselmaier, Jochen Brasch, Thomas Schwarz
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 57 Issue 2 Pg. 361-4 (Aug 2007) ISSN: 1097-6787 [Electronic] United States
• PMID: 17467852 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Monoclonal
• Antibodies, Monoclonal, Murine-Derived
• Glucocorticoids
• Immunologic Factors
• Interleukin 1 Receptor Antagonist Protein
• Receptors, Interleukin-1
• Rituximab
• Prednisolone

Topics

• Antibodies, Monoclonal (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived
• Drug Therapy, Combination
• Glucocorticoids (therapeutic use)
• Humans
• Immunologic Factors (therapeutic use)
• Injections, Subcutaneous
• Interleukin 1 Receptor Antagonist Protein (administration & dosage, therapeutic use)
• Male
• Middle Aged
• Prednisolone (therapeutic use)
• Receptors, Interleukin-1 (antagonists & inhibitors)
• Retreatment
• Rituximab
• Schnitzler Syndrome (drug therapy, pathology)
• Time Factors
• Treatment Failure
• Treatment Outcome