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INTRODUCTION

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Familial Mediterranean fever (FMF) is the prototype of a group of inherited diseases (Table 392-1) 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. 137). 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. Although the hereditary recurrent fevers comprise a major category of the autoinflammatory diseases, other inherited disorders of inflammation in which recurrent fever plays a less prominent role are now also considered to be autoinflammatory.

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Table Graphic Jump Location
TABLE 392-1The Hereditary Recurrent Fever Syndromes
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BACKGROUND AND PATHOPHYSIOLOGY

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FMF was first ...

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