Autoinflammatory diseases: the hereditary periodic fever syndromes

Autoinflammatory diseases: the hereditary periodic fever syndromes

Authors

  • P. Fietta

Keywords:

Human autoinflammatory diseases, hereditary periodic fever syndromes, familial Mediterranean fever, hyperimmunoglobulinemia D and periodic fever syndrome, tumor necrosis factor receptor superfamily 1A-associated periodic syndrome

Abstract

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 syndrome (HIDS), and tumor necrosis factor receptor superfamily 1A-associated periodic syndrome (TRAPS). FMF and HIDS are autosomal recessive diseases, while TRAPS is dominantly inherited. Although each HPFS presents genetic and phenotypic peculiarities, globally these affections share an intermittent expression, in form of acute attacks of fever variably associated with serosal, synovial and/or cutaneous inflammation, usually self-limiting. Amyloidosis is the most severe, life-threatening complication of FMF and TRAPS, whereas it has not been till now reported in HIDS. The HPFS molecular bases have been recently identified. In this paper, the most recent information on HPFSs is reviewed and summarized.

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Published

01-08-2004

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Section

REVIEWS

How to Cite

1.
Autoinflammatory diseases: the hereditary periodic fever syndromes. Acta Biomed [Internet]. 2004 Aug. 1 [cited 2024 Jun. 12];75(2):92-9. Available from: https://www.mattioli1885journals.com/index.php/actabiomedica/article/view/2090