Familial Mediterranean fever and the other autoinflammatory syndromes: evaluation of the patient with recurrent fever.
Purpose Of Review: The aim of this article is to summarize recent clinical, genetic and pathophysiologic findings of familial Mediterranean fever and several of the other systemic autoinflammatory diseases, a recently recognized group of disorders characterized by seemingly unprovoked inflammation b...
| Publicado en: | Current Opinion in Rheumatology Vol. 18; no. 1; pp. 108 - 118 |
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| Autores principales: | , , , |
| Formato: | review tables/charts Journal Article |
| Publicado: |
Lippincott Williams & Wilkins
Jan2006
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106274823&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 106274823 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 10408711 IR0 jtl: Current Opinion in Rheumatology issn: 10408711 maglogo: N pubinfo: dt: Jan2006 vid: 18 iid: 1 pid: 433 pub: Lippincott Williams & Wilkins place: Baltimore, Maryland artinfo: ui: 106274823 106274823 NLM16344627 2009531553 10.1097/01.bor.0000198006.65697.5b NLM16344627 106274823 ppf: 108 ppct: 10 formats: tig: atl: Familial Mediterranean fever and the other autoinflammatory syndromes: evaluation of the patient with recurrent fever. aug: au: Samuels J Ozen S Samuels, Jonathan Ozen, Seza affil: Department of Rheumatology, Hospital for Special Surgery, New York, NY 10021, USA sug: subj: Autoimmune Diseases Diagnosis Fever Etiology Hereditary Diseases Diagnosis Acne Vulgaris Familial and Genetic Algorithms Arthritis Familial and Genetic Autoimmune Diseases Familial and Genetic Autoimmune Diseases Immunology Autoimmune Diseases Physiopathology Carrier Proteins Fever Familial and Genetic Fever Immunology Hereditary Diseases Immunology Hereditary Diseases Physiopathology Hypergammaglobulinemia Immunology Immunoglobulins Immunology Pyoderma Gangrenosum Familial and Genetic Receptors, Cell Surface Immunology Recurrence ab: Purpose Of Review: The aim of this article is to summarize recent clinical, genetic and pathophysiologic findings of familial Mediterranean fever and several of the other systemic autoinflammatory diseases, a recently recognized group of disorders characterized by seemingly unprovoked inflammation but lacking high-titer autoantibodies. Genetic and clinical tools are improving the ability of the clinician to better approach patients with periodic fever and inflammation.Recent Findings: The spectrum of reported genetic mutations and susceptible ethnicities for the hereditary periodic fever subset of the autoinflammatory diseases has continued to expand. At the same time, the pathogeneses of many of these diseases are now understood to involve different aspects of a common pathway, largely affecting inflammatory cascades related to IL-1 or tumor necrosis factor-alpha. Three of these diseases which have been grouped as the cryopyrin-associated periodic syndromes result from defects in the same gene, and all three appear to respond well to anti-IL-1 therapy although controlled trials are still in progress. In addition, cytokine-based therapies are also now under investigation for hyperimmunoglobulinemia D with periodic fever syndrome and pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome.Summary: The identification of the genes and proteins mutated in many of the autoinflammatory diseases has broadened our understanding of the regulation of inflammation and the immune system, and provided the basis for the use of targeted therapies in these syndromes. We propose an algorithm for the evaluation of a patient with periodic fever, taking into account the patient's age, ethnicity, symptoms and signs, and results from laboratory and genetic testing. pubtype: Academic Journal doctype: review tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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