This is an inherited condition of unknown aetiology. It is characterized be episodes of fever, peritonitis and/or pleuritis.
Amyloidosis, skin lesions and arthritis may be seen in these patients.
This condition occurs most often in non-Ashkenazi Jews, Armenians and Arabs. It is not restricted to these groups and may occur in, among others, people of Ashkenazi Jewish, Italian, and Anglo-saxon descent.