Familial Mediterranean fever (a periodic disease): The present-day view of the problem
| Autor: | Evgeny Stanislavovich Fedorov, S O Salugina, N N Kuzmina |
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| Jazyk: | ruština |
| Rok vydání: | 2013 |
| Předmět: | |
| Zdroj: | Современная ревматология, Vol 0, Iss 1, Pp 24-30 (2013) |
| Druh dokumentu: | article |
| ISSN: | 1996-7012 2310-158X |
| Popis: | The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF)/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance. The most common types of mutations are given. Hyperactivation of innate (antigen-specific) immunity is a basic pathogenic mechanism of the disease and IL-1ß is a leading mediator. FMF prominently occurs in certain ethnic groups (Sephardic Jews, Armenians, Turks, and Arabs). In spite of the fact that there may be multiple organ failure, 12-72-hour febrile fever episodes accompanied by the symptoms of peritonitis and/or pleuropericarditis. AA amyloidosis is the most serious complication of FMF. Colchicine therapy is a basic treatment for preventing this complication. In case of colchicine inef-fi-cacy/intolerance, other agents, including genetically engineered biological drugs (IL-1ß inhibitors, etc.), may be used. |
| Databáze: | Directory of Open Access Journals |
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