When amyloidosis was observed in familial Mediterranean fever (1), it seemed obvious to regard it as another example of "secondary" amyloidosis. On closer examination, however, doubts arose. There is, to be sure, the clinical association with attacks of familial Mediterranean fever, but on autopsy, only amyloidosis was found (2-6). A pathologist, lacking clinical information, would classify such a case as "primary," not "secondary," amyloidosis. This alone would be a unique feature, and ought to place the amyloidosis of familial Mediterranean fever in a special category.
The appearance of amyloidosis in familial Mediterranean fever is strangely independent of the clinical attacks