ABSTRACT
A 13-year-old Sephardic Jewish boy had recurrent fever, abdominal pain, arthritis, pleuritic chest pain, and the skin lesions shown here. What is the treatment of choice?
a. Nafcillin b. Cephalexin c. Colchicine d. Prednisone e. Acetaminophen
• FMF is an autosomal recessive disease that affects mostly patients of non-Ashkenazi (e.g., Sephardic) Jewish, Armenian, Turkish, or Arabic descent
• FMF is marked by recurrent paroxysmal episodes lasting 12 to 72 hours involving inflammation of serosal tissues (e.g., pleura, peritoneum, and synovium). The commonly reported symptoms include fever (96%-100%), abdominal pain (89%- 96%), chest pain (33%-57%), arthritis or arthralgias (21%-76%), erysipelas-like rash (12%-41%), and amyloidosis (2%)
• Laboratory abnormalities during attacks often include increased sedimentation rate, increased leukocyte count (neutrophilic predominance), increased fibrinogen and other acute-phase reactants, and microscopic hematuria and proteinuria. These abnormalities usually resolve after the attack
• Onset usually is before age 20 years, but late occurrences have been reported
• The predominant gene mutation is thought to have originated from a single common ancestor who lived about 2,500 years ago, before the Babylonian captivity of the ancient Jews
• Colchicine prophylaxis (1-2 mg per day) greatly reduces the number of attacks and may eliminate them altogether. This therapy has been instrumental in decreasing the frequency of amyloidosis in FMF
