Successful use of anakinra to treat refractory Schnitzler's syndrome.
نویسندگان
چکیده
Schnitzler's syndrome is a rather rare disease which may appear in a rheumatologist's office because patients often report rheumatic symptoms with joint, bone and muscle pain. However, it is characterized by chronic urticaria, recurrent fever, liver and spleen enlargement, osteosclerosis, and lymphadenopathy, in conjunction with a serum IgM M component. A patient who had been treated with relatively high doses of corticosteroids for 10 years with insufficient response was treated with the IL-1 receptor antagonist anakinra, this led to a complete resolution of symptoms.
منابع مشابه
A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra
Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became ...
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BACKGROUND Schnitzler's syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis, and bone pain. The cause and treatment are still unknown. OBJECTIVE To assess treatment with thalidomide and an interleukin 1 receptor antagonist, anakinra, in Schnitzler's syndrome. CASE REPORTS Three patien...
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UNLABELLED Schnitzler's syndrome is a rare disease characterized by a monoclonal IgM (or IgG) paraprotein, a nonpruritic urticarial skin rash, and 2 (or 3) of the following: recurrent fever, objective signs of abnormal bone remodeling, elevated CRP level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. It responds well to treatment with the interleukine-1-inhibitor anakinra. We re...
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We report the case of a 63-year-old male diagnosed with chronic autoimmune mediated meningoencephalitis and periodic cyclical fever syndrome. After 2 years of failed conventional treatment and recurrent hospitalizations, anakinra treatment (an interleukin-1 [IL-1] receptor antagonist) was trialed which resulted in full recovery. As evidenced by this case, anakinra can be used to treat chronic a...
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It is currently unknown if the increase of the hepatic iron regulatory hormone hepcidin during inflammation in man depends on an intact HFE-protein. Here we describe the temporal relationship of serum hepcidin, serum iron and cytokines in a patient with HFE-related (C282Y homozygous) hereditary hemochromatosis who was treated for an auto-inflammatory condition, i.e. variant Schnitzler's syndrom...
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عنوان ژورنال:
- Clinical and experimental rheumatology
دوره 26 2 شماره
صفحات -
تاریخ انتشار 2008