Familial Mediterranean Fever

Episodic bouts of acute peritonitis associated with serositis involving the joints and pleura

Rare autosomal recessive disorder

Almost exclusively affects people of Mediterranean ancestry, especially Sephardic Jews, Armenians, Turks, and Arabs

Patients lack a protease in serosal fluids that normally inactivates interleukin-8 and complement factor 5A

Symptom onset before the age of 20

Fever

Severe abdominal pain and abdominal tenderness with guarding or rebound tenderness

Joint pain, inflammation

Pleuritic chest pain, pleural effusion

Secondary amyloidosis (renal, hepatic) occurs in 25% of cases and can lead to death

Usually a clinical diagnosis

Gene responsible for familial Mediterranean fever (MEFV) has been identified

Genetic tests fail to identify known gene mutation in one-third of patients

Untreated, attacks resolve within 24–48 hours

Patients may undergo unnecessary exploratory laparotomy

Colchicine, 0.6 mg two or three times daily orally

• Decreases the frequency and severity of attacks

• Can prevent or arrest amyloidosis