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Harrison's Principles of Internal Medicine, 18e
| Part 15. Disorders of the Joints and Adjacent Tissues > Section 2. Disorders of Immune-Mediated Injury > |
Chapter 330. Familial Mediterranean Fever and Other Hereditary Recurrent Fevers
Sections: Familial Mediterranean Fever and Other Hereditary Recurrent Fevers: Introduction, Background and Pathophysiology, Acute Attacks, Amyloidosis, Diagnosis, Other Hereditary Recurrent Fevers, Further Readings.
Topics Discussed: familial mediterranean fever; hereditary autoinflammatory diseases.
Excerpt:"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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