Management of familial Mediterranean fever
- Eldad Ben-Chetrit, MD
Eldad Ben-Chetrit, MD
- Professor of Medicine
- Hadassah-Hebrew University Medical School
- Section Editors
- David S Pisetsky, MD, PhD
David S Pisetsky, MD, PhD
- Section Editor — Lupus
- Professor of Medicine and Immunology
- Duke University Medical Center
- J Thomas Lamont, MD
J Thomas Lamont, MD
- Editor-in-Chief — Gastroenterology/Hepatology
- Section Editor — Anorectal Disorders and Misc. Lower GI Disease
- Section Editor — Nutrition, Malabsorption, and Misc. Upper GI Disease
- Professor of Medicine
- Harvard Medical School
Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder characterized by recurrent bouts of fever and serosal inflammation. Its major complication is the insidious development of secondary (AA) amyloidosis with eventual renal failure in uncontrolled patients. This topic will review the management of FMF. The epidemiology, genetics, pathophysiology, and clinical manifestations of FMF and an overview of periodic fever syndromes and other autoinflammatory diseases can be found elsewhere. (See "Familial Mediterranean fever: Epidemiology and pathogenesis" and "Clinical manifestations and diagnosis of familial Mediterranean fever" and "Periodic fever syndromes and other autoinflammatory diseases: An overview".)
The goals of therapy for familial Mediterranean fever (FMF) are to prevent acute attacks and minimize subclinical inflammation in between attacks, and to prevent the development and progression of amyloidosis. Initial treatment of FMF is with colchicine.
Colchicine — Colchicine is primarily effective as a prophylactic treatment for the FMF attacks. It is recommended in all patients regardless of the frequency and intensity of attacks. Use of intermittent high-dose colchicine only for treatment of acute attacks of FMF does not protect against the development of amyloidosis resulting from low-grade inflammation that can occur during asymptomatic intervals [1-5].
Dosing and administration — Treatment with colchicine should be started as soon as a clinical diagnosis is established and should be continued indefinitely. However, in rare cases of heterozygous FMF patients who are asymptomatic for several (more than five) years and do not display elevated acute phase reactants, it may be possible to discontinue colchicine [6,7].
We recommend the following starting dose of colchicine :
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- INITIAL MANAGEMENT
- - Dosing and administration
- - Monitoring
- - Mechanism of action
- - Efficacy
- Decreasing the frequency of attacks
- Prevention of amyloidosis
- - Safety
- SUBSEQUENT MANAGEMENT
- Colchicine-resistance or intolerance
- - Anti-interleukin-1 therapy
- - Other agents
- MANAGEMENT OF SPECIFIC FEATURES
- Chronic arthritis
- Febrile myalgia
- Exertional myalgia
- SPECIAL POPULATIONS
- SUMMARY AND RECOMMENDATIONS