| Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler's syndrome: a case report and literature review. | |
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MedLine Citation:
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PMID: 20119842 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We report a case of longstanding multidrug resistant Schnitzler's syndrome that finally went into clinical remission upon treatment with anakinra and review the literature concerning IL1-RA treatment for Schnitzler's syndrome. A now 71-year-old patient presenting with recurring episodes of urticaria and fever and secondary weight loss for the past 16 years as well as arthralgia, hearing loss. The patient has anemia, leucocytosis with neutrophilia, thrombocytosis, elevated C-reactive protein and erythrocyte sedimentation rate, lymphadenopathy and a monoclonal IgM kappa band that later became oligoclonal with two IgM kappa bands and one IgM lambda band. The patient was treated with moderate effect with combination of prednisolone, azathioprine, and colchicine. In March 2009, anakinra 100 mg daily sc was added during a disease flare. Within 24 h after the first injection, both the urticaria and the fever disappeared and have not recurred. For the past 6 months, the patient has been in clinical and biochemical remission. Colchicine has been stopped while the azathioprine and prednisolone doses are being reduced. Twenty-four patients with Schnitzler's syndrome, including three patients with a variant of Schnitzler's syndrome and three patients with a Schnitzler-like syndrome, have been successfully treated with anakinra. Nevertheless, seven out of seven patients, that either interrupted or used anakinra every other day, had relapse of their symptoms within 24-48 h; anakinra was restarted in all patients with the same clinical efficiency. The current case history and the literature review already suggest an important role for IL-1 as a mediator in the pathophysiology of Schnitzler's syndrome. |
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Authors:
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Emilio Besada; Hans Nossent |
Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-02-01 |
Journal Detail:
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Title: Clinical rheumatology Volume: 29 ISSN: 1434-9949 ISO Abbreviation: Clin. Rheumatol. Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-03-23 Completed Date: 2010-07-28 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8211469 Medline TA: Clin Rheumatol Country: Germany |
Other Details:
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Languages: eng Pagination: 567-71 Citation Subset: IM |
Affiliation:
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Rheumatology, University Hospital North Norway, Tromsø, Norway. emilio.besada@unn.no |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Azathioprine / pharmacology Blood Sedimentation C-Reactive Protein / metabolism Colchicine / pharmacology Female Humans Immunoglobulin G / immunology Leukocytosis / immunology Male Middle Aged Paraproteinemias / pathology Prednisolone / pharmacology Receptors, Interleukin-1 Type I / metabolism* Schnitzler Syndrome / immunology*, therapy Time Factors Urticaria / immunology |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulin G; 0/Receptors, Interleukin-1 Type I; 446-86-6/Azathioprine; 50-24-8/Prednisolone; 64-86-8/Colchicine; 9007-41-4/C-Reactive Protein |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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