$Unique_ID{BRK03982} $Pretitle{} $Title{Mediterranean Fever, Familial} $Subject{Mediterranean Fever, Familial Siegel-Cattan-Mamou Syndrome Armenian Syndrome Benign Paroxysmal Peritonitis Familial Paroxysmal Polyserositis Mediterranean Fever Periodic Amyloid Syndrome Periodic Peritonitis Syndrome Recurrent Polyserositis Reimann's Syndrome Reimann Periodic Disease } $Volume{} $Log{} Copyright (C) 1986, 1990, 1992 National Organization for Rare Disorders, Inc. 127: Mediterranean Fever, Familial ** IMPORTANT ** It is possible that the main title of the article (Familial Mediterranean Fever) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Siegel-Cattan-Mamou Syndrome Armenian Syndrome Benign Paroxysmal Peritonitis Familial Paroxysmal Polyserositis Mediterranean Fever Periodic Amyloid Syndrome Periodic Peritonitis Syndrome Recurrent Polyserositis Reimann's Syndrome Reimann Periodic Disease General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section. Familial mediterranean fever (FMF) is a hereditary disorder characterized by recurrent bouts of fever and inflammation of the serous membranes, which line the inside of the body cavity and the various organs that protrude into it. FMF occurs in persons of Armenian, Arabic, or Sephardic Jewish ancestry, and affects males more often than females. The disorder has a good prognosis in the absence of complications. The causes of Familial Mediterranean Fever are not known. Symptoms The first symptoms of Familial Mediterranean Fever usually appear between the ages of five and fifteen, although onset occasionally occurs in young adulthood or early middle age. Attacks recur at intervals of weeks or months, and usually last twenty-four to forty-eight hours, although sometimes they can last as long as one week. In women, attacks may correspond with menstrual periods. Fever, sometimes severe abdominal and stabbing chest pain (due to inflammation of the pleural and peritoneal membranes), and occasionally, arthritis and skin lesions, characterize the attacks. The patient may feel unable to breathe deeply because of pain (pleurisy). Pain occurs in the larger joints and lasts 2 to 3 days. Skin lesions consist of painful, reddish, swollen areas, usually on the lower legs. A major complication of Familial Mediterranean Fever is amyloidosis particularly affecting the kidneys. (For more information on Amyloidosis, choose "amyloidosis" as your search term in the Rare Disease Database.) This can lead to kidney failure. In the United States, drug addiction is an important complication, usually resulting from the use of narcotics to alleviate pain during attacks. Causes Familial Mediterranean Fever is inherited through an autosomal recessive mechanism. (Human traits including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In recessive disorders, the condition does not appear unless a person inherits the same defective gene from each parent. If one receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will show no symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is twenty-five percent. Fifty percent of their children will be carriers, but healthy as described above. Twenty-five percent of their children will receive both normal genes, one from each parent and will be genetically normal.) Scientists have located the gene that results in the development of Familial Mediterranean Fever in non-Ashkanazi Jews on the short arm of chromosome 16. The biochemical or structural defect in this disease is not known. Inherited metabolic or endocrine defects are possibilities. Affected Population Familial Mediterranean Fever preferentially affects persons of Armenian, Arabic, or Sephardic Jewish ancestry. More males than females are affected. Therapies: Standard Colchicine prevents attacks for reasons that are not understood. In larger quantities, it stops attacks that are already in progress. Narcotics should not be used routinely to control pain because of the possibility of drug addiction. Surgery is of no benefit. When amyloidosis has destroyed the kidneys, renal transplantation or renal dialysis may be necessary. Therapies: Investigational This disease entry is based upon medical information available through June 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Familial Mediterranean Fever, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Institute of Diabetes, Digestive & Kidney Diseases Information Clearinghouse Box NDIC Bethesda, MD 20892 (301) 468-2162 For information on genetics and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 147, 795, 1196-9. THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 255.