Familial Mediterranean fever (FMF) is the prototype of a group of inherited diseases (Table 330-1) that are characterized by recurrent episodes of fever with serosal, synovial, or cutaneous inflammation and, in some individuals, the eventual development of systemic AA amyloidosis (Chap. 112). Because of the relative infrequency of high-titer autoantibodies or antigen-specific T cells, the term autoinflammatory has been proposed to describe these disorders, rather than autoimmune. The innate immune system, with its myeloid effector cells and germline receptors for pathogen-associated molecular patterns and endogenous danger signals, plays a predominant role in the pathogenesis of the autoinflammatory diseases.

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Table 330-1 The Hereditary Recurrent Fever Syndromes 

FMF was first recognized among Armenians, Arabs, Turks, and non-Ashkenazi (primarily North African and Iraqi) Jews. With the advent of genetic testing, FMF has been documented with increasing frequency among Ashkenazi Jews, Italians, and other Mediterranean populations, and occasional cases have been confirmed even in the absence of known Mediterranean ancestry. FMF is recessively inherited, but, particularly in countries where families are small, a positive family history ...

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