Partial response to cyclosporine in a patient with Schnitzler's syndrome.
Schnitzler's syndrome is an unusual clinical association of chronic urticaria, intermittent fever and monoclonal immunoglobulin M (IgM) gammopathy. The pathogenesis of the urticaria is unclear and treatment is problematic. We describe the case of a 61-year-old woman with a long history of chronic urticaria with severe pruritus, spiking fever and malaise. The IgM-kappa monoclonal component was detected in the patient's serum 4 years after symptom onset. After ineffective treatment with antihistamines and systemic corticosteroids, oral cyclosporine resulted in complete remission of the fever and malaise, which has persisted after an 18-month follow-up. Partial but maintained remission of the urticaria was also observed, allowing corticosteroid doses to be decreased.
Carbone
J
J
Clinical Immunology Unit, Immunology Department, University Hospital Gregorio Marañon, Madrid, Spain. carbone@teleline.es
Paravisini
A
A
Sarmiento
E
E
Rodríguez-Molina
J
J
Fernández-Cruz
E
E
D018771:Arthralgia / Q000209:etiology; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005334:Fever / Q000209:etiology; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D010266:Paraproteins / Q000032:analysis; D012008:Recurrence; D019873:Schnitzler Syndrome / Q000097:blood / Q000188:drug therapy*; D014581:Urticaria / Q000188:drug therapy / Q000209:etiology
D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D007166:Immunosuppressive Agents; D010266:Paraproteins; D016572:Cyclosporine; D008775:Methylprednisolone