Familial Mediterranean fever

(peritonitis, episodic fever, pleuritis; ↑ WBC & ESR during attacks);

• Recessive MEFV mutations (activating pyrin, upstream of inflammasome)
• Febrile episodes for 1–3 d w/ abd pain (serositis, can mimic acute abdomen), pleurisy, lg joint monoarthritis/arthralgia, erysipelas-like skin lesions. Variable time between attacks.
• Epidemiology: ↑ prev in Armenians (1/500), Sephardic Jews, North Africans, Turks, Arabs; onset usually as child
• Risk of ESRD due to amyloidosis (AA), peritoneal adhesions, or infertility if untreated
• Colchicine effective prophylactic and ↓ amyloid; anti-IL1 is alternative (NEJM 2018;378:1908)