Archives of Internal Medicine · 1961 · 33 citations · 8 references
Life SciencesGeneticsPathologyAutoimmunityHistopathologyInflammationAutoimmune Disease Susceptibility GenesAutoimmune DiseasesGenetic DisordersInherited Disease DiagnosisInherited Disease GeneticsInherited Metabolic Disease
Familial Mediterranean fever is an inherited disease in persons of Mediterranean ancestry.<sup>1</sup>The illness is characterized by short, self-limited attacks of fever, usually accompanied by severe pain in the abdomen or chest. Familial Mediterranean fever is also known as periodic disease,<sup>2,3</sup>benign paroxysmal peritonitis,<sup>4</sup>the Armenian's disease,<sup>5</sup>and familial recurring polyserositis.<sup>6</sup>There is little doubt that this disorder is an inborn error of metabolism, and in certain susceptible populations, such as the Jews of Iraq, it has been calculated that familial Mediterranean fever is determined by a recessive gene present in approximately 1 in 52 persons, with the manifest illness appearing in a ratio of about 1 to 2,720.<sup>7</sup>The only demonstrable histopathology, other than a nonspecific inflammatory response, such as peritonitis, during the attacks of fever, has been the emergence of amyloidosis in a high percentage of the patients.<sup>8</sup>The underlying metabolic
8
Harry Heller · Archives of Internal Medicine · 1958 · 277 citations
Sheppard Siegal · Annals of Internal Medicine · 1945 · 197 citations
Hobart A. Reimann · Journal of the American Medical Association · 1948 · 131 citations
Public Health, Infectious Diseases, Clinical Epidemiology +6