Familial Mediterranean Fever (redirected from familial paroxysmal polyserositis)

familial Mediterranean fever [fə′mil·yəl ¦med·ə·tə¦rā·nē·ən ¦ fē·vər]

(medicine)

A hereditary disease of unknown cause characterized by recurrent fever, abdominal and chest pain, arthralgia, and rash, sometimes terminating in renal failure. Abbreviated FMF. Also known as familial recurring polyserositis; periodic disease; periodic peritonitis.

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Familial Mediterranean Fever 

(also benign paroxysmal peritonitis), a recurring, superficial aseptic inflammation of the serous membranes of the pleural part of the peritoneum, accompanied by heavy exudation. In most cases, the. disease arises before the age of 30. It is assumed to be a hereditary, recessive disturbance of metabolism that is probably autosomal; although men are affected more often than women, no sex-linkage has been shown. Other types of disturbances, including diencephalic and endocrine, are possibly involved.

The clinical picture of familial Mediterranean fever can take four forms. The abdominal form entails symptoms of acute abdomen, as a result of which almost half the patients with this form must undergo surgery. The thoracic form proceeds with a picture of dry or exudative pleuritis and with exudation inside the sinuses. The articular form resembles arthralgia or arthritis that affects one or more joints, and the remaining form is pseudomalarial.

Characteristic of all forms of familial Mediterranean fever are the chronic, recurring course and the serious condition during attacks that alternates with a sensation of complete health during the remissions of various duration. The attacks are typical and differ from each other only in severity and duration. They usually last from one to four days and leave no observable anatomical changes. All cases show similar nonspecific shifts in laboratory-test parameters, and the possibility of developing amyloidosis arises with 30–43 percent of patients. The amyloidosis leads to renal insufficiency and is associated neither with the frequency or character of the attacks nor with the duration of the disease. Treatment has little effect on the course of familial Mediterranean fever.

REFERENCES

Oganesov, L. A., and V. M. Avakian. “Riad sluchaev neizvestnoi infektsionnoi bolezni.” Sovetskaia meditsina, 1938, no. 16, pp. 9–12.
Vinogradova, O. M. Periodicheskaia bolezn’. Moscow, 1973.
Siegal, S. “Benign Paroxysmal Peritonitis.” Annals of Internal Medicine, 1945, vol. 23, no. 1, pp. 1–21.
Siegal, S. “Benign Paroxysmal Peritonitis—Second Series.” Gastroenterology, 1949, vol. 12, no. 2. pp. 234–47.

O. M. VINOGRADOVA