Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Familial Mediterranean Fever (FMF) is a hereditary inflammatory disorder that affects groups of people originating from around the Mediterranean Sea (hence its name). It is prominently present in the Armenian people, Sephardi Jews (and, to a much lesser extent, Ashkenazi Jews), people from Turkey, and the Arab countries.[1]
There are seven types of attacks. 90% of all patients have their first attacks before they are 18 years old. All develop over 2-4 hours and last anytime between 6 hours and 4 days. Most attacks involve fever:[1]
AA-amyloidosis with renal failure is a complication and may develop without overt crises. AA (amyloid protein) is produced in very large quantities during attacks and at a low rate between them, and accumulates mainly in the kidney, as well as the heart, spleen, gastrointestinal tract and the thyroid.[1]
There appears to be an increase in the risk for developing particular vasculitis-related diseases (e.g. Henoch-Schönlein purpura), spondylarthropathy, prolonged arthritis of certain joints and protracted myalgia.[1]
The diagnosis is clinically made on the basis of the history of typical attacks, especially in patients from the ethnic groups in which FMF is more highly prevalent. An acute phase response is present during attacks, with high C-reactive protein levels, an elevated white blood cell count and other markers of inflammation. In patients with a long history of attacks, monitoring the renal function is of importance in predicting chronic renal failure.[1]
A genetic test is also available now that the disease has been linked to mutations in the MEFV gene. Sequencing of exons 2, 3, 5, and 10 of this gene detects an estimated 97% of all known mutations.[1]
[tubepress mode=’tag’, tagValue=’Familial Mediterranean fever’]