Familial Mediterranean Fever

We had a patient with suspected familial Mediterranean fever down for a CT tonight. Luckily, Zissin et al had a nice review of the CT features of familial Mediterranean fever in the BJR.

Familial Mediterranean fever, also known as recurrent polyserositis, is an autosomal recessive condition that tends to occur in ethnic groups originating in the Mediterranean area: Sephardic Jews, Arabs, Turks and Armenians. Diagnosis requires genetic tests.

Patients typically present before the age of 20 with repeated, self-limited episodes of severe, diffuse abdominal pain and rigidity, and fever due to inflammation of serous (e.g., peritoneum) surfaces. Inflammation of synovial surfaces can also occur, leading to joint pain.

A minority of patients present with surgical emergencies (e.g., acute appendicitis or complicated small bowel obstruction). The remainder present with mild, non-surgical peritonitis characterized by nonspecific CT findings of mesenteric edema: Edematous and hyperemic peritoneal folds and greater omentum with vascular markings, interenteric exudates and fibrinonodular changes. Mesenteric lymphadenopathy and splenomegaly have also been reported.

Our patient ended up not having much of anything on CT.

References

Zissin R, Rathaus V, Gayer G, Shapiro-Feinberg M, Hertz M. CT findings in patients with familial Mediterranean fever during an acute abdominal attack. Br J Radiol. 2003 Jan;76(901):22-5.