Familial Mediterranean Fever


Also found in: Dictionary, Thesaurus, Medical, Legal, Acronyms, Wikipedia.

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.

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

References in periodicals archive ?
Colchicine treatment in conception and pregnancy: two hundred thirty-one pregnancies in patients with familial Mediterranean fever.
Familial mediterranean fever as an emerging clinical model of atherogenesis associated with low-grade inflammation.
The frequency of sacroiliitis in familial Mediterranean fever and the role of HLA-B27 and MEFV mutations in the development of sacroiliitis.
Bone mineral density in patients with familial Mediterranean fever.
Familial Mediterranean fever in Crete: a genetic and structural biological approach in a population of intermediate risk.
Table 1: Immunoglobulin light chain levels in patients with juvenile idiopathic arthritis, familial mediterranean fever and in healthy controls and their comparison with each other (Data are given as median (range); p < 0.
2,3) Ethnicity (regions where familial Mediterranean fever is prevalent), chronic inflammations, infections (such as tuberculosis), rheumatic diseases, and obstructive lung disease were important etiologic factors in our study.
The situation started last summer when the Food and Drug Administration approved URL's colchicine (Colcrys) for gout and familial Mediterranean fever.
The spectrum of MEFV clinical presentations--is it familial Mediterranean fever only?
Several rheumatologic pathologies have been reported to coexist with OPK, such as rheumatoid arthritis, lupus erythematosus, reactive arthritis, ankylosing spondylitis and familial Mediterranean fever (13,18-23) but these were not seen in our patient.

Full browser ?