Thursday, May 10, 2012


An Armenian patient comes to you in desperation because "no doctors have been able to figure out what is wrong with me." He says his first attack began in childhood, before adolescence. He had severe belly pain and fever for 2-3 days which got better on its own. Since then, he's had recurrent attacks of abdominal pain and fever with an abrupt onset, lasting 1-3 days, resolving spontaneously. In between attacks he feels perfectly well. He has not been able to identify any triggers.

The abdominal pain usually starts with focal pain and tenderness that then generalizes with guarding, rebound tenderness, rigidity, and ileus. In fact, he's had an exploratory laparotomy in the past, but the surgeons found nothing remarkable and took out a perfectly normal appendix. Once, he had unilateral pleuritic chest pain and a pleural effusion that resolved after several days. During these attacks, laboratory tests show leukocytosis with neutrophillia and elevated acute phase reactants like ESR and CRP.

Challenge: Although these short term attacks cause a great deal of morbidity, the major source of mortality is progressive secondary amyloidosis. What is this?

Image of map is in the public domain, from Wikipedia.


Reflex Hammer said...

Familial Mediterranean Fever!

Craig Chen said...

ooh nice one! i've never seen this disease, but it's always on the tests

Familial Mediterranean fever is an autosomal recessive disorder characterized by sporadic paroxysmal attacks of fever and serosal inflammation, seen mostly in Sephardic Jews, Armenians, Turks, North Africans, and Arabs.

Source: UpToDate.