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An Update on Familial Mediterranean Fever

Maddalena Lancieri1, Marta Bustaffa1, Serena Palmeri1

  • 1UOC Malattie Autoinfiammatorie e Immunodeficenze, IRCCS Istituto Giannina Gaslini, 16147 Genova, Italy.

International Journal of Molecular Sciences
|June 10, 2023
PubMed

Insights

Familial Mediterranean Fever (FMF) is an autoinflammatory disease causing recurrent inflammation. This review updates FMF pathophysiology, genetics, diagnosis, and treatment, including resistance and compliance.

Area of Science:

  • Immunology
  • Genetics
  • Rheumatology

Background:

  • Familial Mediterranean Fever (FMF) is the primary autoinflammatory disorder.
  • Characterized by recurrent neutrophilic inflammation and polyserositis.
  • Associated with severe complications like renal amyloidosis.

Purpose of the Study:

  • To provide an updated overview of FMF.
  • Integrate recent literature with novel insights on treatment resistance and compliance.
  • Clarify pathophysiology, genetics, diagnosis, and treatment.

Main Methods:

  • Literature review of recent studies on FMF.
  • Integration of current data on treatment resistance and patient compliance.
  • Synthesis of information on FMF pathophysiology, genetics, diagnosis, and treatment.

Main Results:

  • FMF typically presents in childhood with fever and polyserositis.
  • Long-term complications include renal amyloidosis.
  • Recent characterization has improved understanding of FMF.

Conclusions:

  • The review offers an updated perspective on FMF.
  • Includes real-life outcomes of treatment resistance recommendations.
  • Enhances understanding of autoinflammatory processes and innate immunity.
Keywords:
Familial Mediterranean FeverInterlukin-1 Inhibitorsautoinflammatory diseasechildrencolchicinecolchicine resistance

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