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