Hereditary Periodic Fever Syndromes: Autoinflammatory Diseases
نویسندگان
چکیده
منابع مشابه
Autoinflammatory 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 کاملPathogenesis of familial periodic fever syndromes or hereditary autoinflammatory syndromes.
Familial periodic fever syndromes, otherwise known as hereditary autoinflammatory syndromes, are inherited disorders characterized by recurrent episodes of fever and inflammation. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to very minor stimuli or turned off too late. The genetic background of the major familial perio...
متن کاملHereditary periodic fever syndromes.
The hereditary periodic fevers are a group of Mendelian disorders characterized by seemingly unprovoked fever and localized inflammation. Recent data indicate that these illnesses represent inborn errors in the regulation of innate immunity. Pyrin, the protein mutated in familial Mediterranean fever, defines an N-terminal domain found in a large family of proteins involved in inflammation and a...
متن کاملHereditary periodic fever syndromes
osting by E Abstract Hereditary periodic fever syndromes, comprise a group of hereditary disorders with similar clinical features of recurrent short episodes of fever associated with inflammatory manifestations. These are usually self-limited in nature and occur in the absence of infection or autoimmune reaction. Between attacks, patients feel well and regain their normal daily functions until ...
متن کاملPeriodic fever syndrome and autoinflammatory diseases
The concept of autoinflammatory disease as a new disease classification has resulted in a paradigm shift in our understanding of the the broad spectrum of immunological diseases. The effectiveness of interleukin-1 blockade in a variety of disorders has resulted in a marked reduction in suffering for many of these patients.
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ژورنال
عنوان ژورنال: Internal Medicine
سال: 2005
ISSN: 0918-2918,1349-7235
DOI: 10.2169/internalmedicine.44.694