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Genetics of Familial Mediterranean Fever and Its Implications
- Philadelphia, PA 19106 Requests for Reprints: George E. Ehrlich, MD, One Independence Place (1101), 241 South Sixth Street, Philadelphia, PA 19106-3731.
In 1945, Dr. Sheppard Siegal, an allergist at Mount Sinai Hospital in New York, reported in Annals five cases of “recurrent paroxysms of severe abdominal pain with fever … as high as 105 °F,” all in men and all starting early in life. He named the syndrome benign paroxysmal peritonitis [1]. The peritoneal signs were so severe that “emergency operation [had] been repeatedly urged.” Pleuritic chest pain often accompanied the attacks. At least one of the patients had “urticarial” lesions near the ankle, and another had intermittent joint pains, usually monoarticular. As an allergist, Siegal approached these cases from his own vantage point, and the history of this condition is replete with references to potential allergens and allergic relatives. Unfortunately, except for noting that all of the patients were white men, he provided no other ethnic background data. Although an occasional similar case was found in previous published series, including some in which such manifestations were interpreted as “Henoch” purpura (from which Siegal clearly differentiated the condition), this landmark article was essentially the first to describe the clinical presentation of what is now known as familial Mediterranean fever.
Dr. Hobart Reimann, then working at the American University in Beirut, had long been collecting information on clinical phenomena of intermittent and periodic character, which …
