Proposed mechanism of the inflammatory attacks in familial Mediterranean fever
Article Abstract:
Familial Mediterranean fever (FMF) is an inherited disease, and although it seems to be similar to autoimmune disorders, the precise pathogenesis is not known. Patients with FMF have inflammation involving the synovium of the joints, the pleural cavities encasing the lungs, and the peritoneal cavity surrounding the abdominal organs. Previous research has indicated that normal individuals have a protein in synovial and peritoneal fluid which inhibits the action of C5a, a protein fragment from the complement system. The complement system normally punches deadly holes in the membranes of invading organisms. In addition, the C5a fragment is a potent stimulator of inflammatory reactions. It was hypothesized that if the C5a fragment is normally inhibited in healthy individuals, then some individuals with inflammatory illnesses might be suffering from a deficiency of this C5a inhibitor. To test this notion, C5a inhibitor was measured in both healthy individuals and people with familial Mediterranean fever. The results indicate that patients with FMF have roughly 1 percent of the normal amount of C5a inhibitor in their peritoneal fluid. It is not clear that the deficiency of C5a inhibitor is a direct result of the gene which causes FMF, however, these findings provide a possible mechanism for the observed inflammatory changes occurring in these patients. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Internal Medicine
Subject: Health
ISSN: 0003-9926
Year: 1990
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Changing the Clinical Management of Hereditary Hemochromatosis: Translating Screening and Early Case Detection Strategies Into Clinical Practice
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Publication Name: Archives of Internal Medicine
Subject: Health
ISSN: 0003-9926
Year: 2004
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