Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease caused by mutations in the MEFV gene, which codes for the Pyrin protein. It typically follows an autosomal recessive inheritance pattern, meaning two abnormal copies of the MEFV gene are required for the disease to manifest. However, in rare cases, especially in children, even a single mutated gene copy can lead to the disease, or symptoms may occur even without detectable genetic mutations.

The Pyrin protein, produced by the MEFV gene, plays a critical role in the natural control of inflammatory processes. When this gene does not function properly, the inflammatory response becomes uncontrolled, leading to recurrent episodes of fever and pain without infection.

Factors that may increase the risk of Familial Mediterranean Fever include:

* Family History: Individuals with a family history of FMF have a higher risk of developing the disease.
* Mediterranean Ancestry: Having an ethnic background specific to the Mediterranean region can increase the likelihood of developing FMF.