Abstract | 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: 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.
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Authors | Jonathan Samuels, Seza Ozen |
Journal | Current opinion in rheumatology
(Curr Opin Rheumatol)
Vol. 18
Issue 1
Pg. 108-17
(Jan 2006)
ISSN: 1040-8711 [Print] United States |
PMID | 16344627
(Publication Type: Journal Article, Review)
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Chemical References |
- Carrier Proteins
- Immunoglobulin D
- NLR Family, Pyrin Domain-Containing 3 Protein
- NLRP3 protein, human
- Receptors, Tumor Necrosis Factor
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Topics |
- Acne Vulgaris
(genetics)
- Algorithms
- Arthritis
(genetics)
- Autoimmune Diseases
(diagnosis, genetics, immunology, physiopathology)
- Carrier Proteins
(genetics)
- Familial Mediterranean Fever
(diagnosis, genetics, immunology, physiopathology)
- Fever
(etiology, genetics, immunology)
- Humans
- Hypergammaglobulinemia
(immunology)
- Immunoglobulin D
(immunology)
- NLR Family, Pyrin Domain-Containing 3 Protein
- Pyoderma Gangrenosum
(genetics)
- Receptors, Tumor Necrosis Factor
(immunology)
- Recurrence
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