Hereditary Autoinflammatory Diseases; Diagnosis and Management
نویسندگان
چکیده
منابع مشابه
[Hereditary systemic autoinflammatory diseases].
Systemic autoinflammatory diseases encompass different rare clinical entities characterized by recurrent acute inflammatory episodes secondary to a dysregulated inflammatory process. Since their first clinical descriptions, the Mendelian hereditary nature of some of them became evident, with their genetic and molecular basis being recently elucidated. There are disease-causing mutations in gene...
متن کاملAutoinflammatory diseases: the hereditary periodic fever syndromes.
Human autoinflammatory diseases (HAIDs) are a heterogeneous group of genetically determined affections characterized by seemingly unprovoked inflammation, in the absence of autoimmune or infective causes. The hereditary periodic fever syndromes (HPFSs) are a HAID subset consisting of three main nosologic entities: familial Mediterranean fever (FMF), hyperimmunoglobulinemia D and periodic fever ...
متن کاملAutoinflammatory syndromes: diagnosis and management
During the last decades the description of autoinflammatory syndromes induced great interest among the scientific community. Mainly rheumatologists, immunologists and pediatricians are involved in the discovery of etiopathogenesis of these syndromes and in the recognition of affected patients. In this paper we will discuss the most important clues of monogenic and non-genetic inflammatory syndr...
متن کاملPediatric hereditary autoinflammatory syndromes.
OBJECTIVE To describe the most prevalent pediatric hereditary autoinflammatory syndromes. SOURCES A review of the literature including relevant references from the PubMed and SciELO was carried out using the keywords autoinflammatory syndromes and child. SUMMARY OF THE FINDINGS The hereditary autoinflammatory syndromes are caused by monogenic defects of innate immunity and are classified as...
متن کاملRecommendations for the management of autoinflammatory diseases
: Autoinflammatory diseases are characterised by fever and systemic inflammation, with potentially serious complications. Owing to the rarity of these diseases, evidence-based guidelines are lacking. In 2012, the European project Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate regimens for the management of children and young ad...
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ژورنال
عنوان ژورنال: Nihon Naika Gakkai Zasshi
سال: 2014
ISSN: 0021-5384,1883-2083
DOI: 10.2169/naika.103.2594