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Therapeutic approach to familial Mediterranean fever: a review update
Mehmet Akif Ozturk1, Mehmet Kanbay, Benan Kasapoglu
1Department of Medicine, Division of Rheumatology, Gazi University School of Medicine, Turkey. makifozturk@yahoo.com
Abstract:
Familial Mediterranean fever (FMF) is a hereditary disorder characterised by recurrent attacks of fever with peritonitis or pleuritis, arthritis, myalgia or erysipelas-like skin lesions. The continuous inflammation in FMF is associated with increased serum amyloid A (SAA) protein which may lead to secondary amyloidosis and deposition of this insoluble protein in the kidney, gut, spleen, liver, heart etc. Therefore, treatment of patients with FMF is beneficial not only for the prevention of the acute attacks but also for improving their prognosis. In the present review we summarise the medical literature concerning FMF treatment, including new therapeutic agents and management of colchicine-resistant patients. Three electronic databases (MEDLINE, EMBASE, and the Cochrane Library) were searched from 1 January 1960 to 28 February 2010 for any therapeutic approach to FMF, with MeSH headings and text words (Familial Mediterranean Fever, FMF treatment, colchicine, infliximab, anakinra, SSRI). In conclusion, colchicine remains the mainstay therapeutic option in FMF. It is effective in various manifestations of the disease such as fever, peritonitis and pleuritis. It prevents the development of amyloidosis. It is safe in humans regarding fertility, and can be used during pregnancy and nursing. Dose adjustment should be made in patients with renal or hepatic failure. It is less effective in arthritis or myalgia, requiring additional treatment with NSAIDs and steroids. In the few cases where FMF is resistant to colchicine other measures, including corticosteroids, non-biological and biological DMARDs, interferon alpha and SSRIs should be employed.
Insights
Familial Mediterranean Fever (FMF) treatment primarily relies on colchicine, which effectively prevents attacks and amyloidosis. For colchicine-resistant cases, alternative therapies like biologics and steroids offer management options.
Area of Science:
- Genetics and Immunology
- Rheumatology
- Internal Medicine
Background:
- Familial Mediterranean Fever (FMF) is an inherited autoinflammatory disorder causing recurrent fever and inflammation.
- Chronic inflammation in FMF elevates serum amyloid A (SAA), potentially leading to secondary amyloidosis in organs.
- Effective FMF treatment is crucial for preventing acute attacks and improving long-term prognosis.
Purpose of the Study:
- To review current medical literature on Familial Mediterranean Fever (FMF) treatment strategies.
- To summarize therapeutic agents, including novel options and management for colchicine-resistant FMF.
- To provide an evidence-based overview of FMF management.
Main Methods:
- Comprehensive literature search of MEDLINE, EMBASE, and Cochrane Library databases (1960-2010).
- Inclusion of studies on Familial Mediterranean Fever (FMF) treatment, colchicine, and alternative therapies like infliximab, anakinra, and SSRIs.
- Analysis of therapeutic approaches for FMF manifestations and resistance.
Main Results:
- Colchicine is the cornerstone of FMF treatment, effectively managing fever, peritonitis, pleuritis, and preventing amyloidosis.
- Colchicine is safe for fertility, pregnancy, and nursing, with dose adjustments needed for renal/hepatic impairment.
- Colchicine shows limited efficacy for arthritis and myalgia, often requiring additional NSAIDs and steroids.
Conclusions:
- Colchicine remains the primary therapy for Familial Mediterranean Fever (FMF), offering significant benefits in disease control and complication prevention.
- For colchicine-resistant FMF, alternative treatments such as corticosteroids, DMARDs (non-biological and biological), interferon alpha, and SSRIs are indicated.
- Comprehensive management strategies are essential for optimizing outcomes in patients with FMF.
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