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Familial Mediterranean fever
1aDepartment of Pediatrics A, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer bDepartment of Pediatrics, Hadassah Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel.
Purpose Of Review:
Familial Mediterranean fever (FMF) is the oldest and most common of the hereditary autoinflammatory diseases (AIDs). A large body of information has been accumulated over recent years on the pathophysiology, diagnosis and treatment of these diseases. The purpose of this review is to bring an up-to-date summary of the clinic manifestations, diagnostic criteria and treatment of FMF.
Recent Findings:
An overview of the pathophysiologic basis of FMF as part of the AID is discussed. Over the last year, attempts to establish new criteria for childhood FMF, new guidelines for treatment and follow-up of disease and novel treatment for FMF were made. A comparison of the different disease severity scores for research purposes suggests that a new score is needed. New evidence for antiinterleukin-1 blockade as a new treatment modality is described.
Summary:
New diagnostic criteria, disease severity score, treatment and follow-up guidelines have been proposed, and need validation in the next several years.
Insights
Familial Mediterranean fever (FMF), a common autoinflammatory disease, has recent updates in diagnosis and treatment. This review summarizes current clinical findings, diagnostic criteria, and management strategies for FMF.
Area of Science:
- Genetics and Immunology
- Autoinflammatory Diseases
- Rheumatology
Background:
- Familial Mediterranean fever (FMF) is the most prevalent hereditary autoinflammatory disease (AID).
- Recent advancements have expanded knowledge on FMF pathophysiology, diagnosis, and treatment.
- This review focuses on providing an updated summary of FMF.
Purpose of the Study:
- To present an up-to-date summary of Familial Mediterranean fever (FMF).
- To review the clinical manifestations, diagnostic criteria, and treatment of FMF.
- To provide an overview of the pathophysiologic basis of FMF within the context of AIDs.
Main Methods:
- Review of recent literature on FMF.
- Analysis of new diagnostic criteria and treatment guidelines.
- Evaluation of disease severity scores and novel therapeutic approaches.
Main Results:
- New criteria for childhood FMF and updated treatment/follow-up guidelines have been proposed.
- A need for a new disease severity score for research purposes is identified.
- Emerging evidence supports anti-interleukin-1 blockade as a novel treatment modality for FMF.
Conclusions:
- Proposed diagnostic criteria, severity scores, and treatment guidelines require further validation.
- Ongoing research aims to refine the management and understanding of FMF.
- Anti-interleukin-1 therapy shows promise for FMF treatment.
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