Related Experiment Videos

Evidence-based recommendations for the practical management of Familial Mediterranean Fever

Véronique Hentgen1, Gilles Grateau, Isabelle Kone-Paut

  • 1Centre national de Référence des maladies Auto-Inflammatoires, CeRéMAI, Centre Hospitalier de Versailles, 177, rue de Versailles, 78150 Le Chesnay Cedex, France.

Seminars in Arthritis and Rheumatism
|June 8, 2013
PubMed
Abstract

Insights

Familial Mediterranean Fever (FMF) management requires colchicine dose adjustment for frequent attacks. For resistant cases, IL1 inhibitors are recommended, and routine genetic screening for asymptomatic siblings is not advised.

Area of Science:

  • Rheumatology
  • Genetics
  • Immunology

Background:

  • Familial Mediterranean Fever (FMF) is a prevalent autoinflammatory disorder characterized by recurrent fevers.
  • Current FMF management faces standardization challenges regarding colchicine therapy and genetic screening.
  • Key issues include colchicine dosage, resistance definition, alternative treatments, and genetic testing for asymptomatic relatives.

Purpose of the Study:

  • To provide consensus recommendations for practical management of FMF patients.
  • To address unresolved issues in daily FMF care, including colchicine use and genetic screening.
  • To standardize approaches to colchicine dosage adjustment, resistance, and alternative therapies.

Main Methods:

  • A comprehensive literature review of PubMed was conducted.
  • Expert recommendations were formulated during a consensus meeting.
  • The review focused on practical management questions in FMF.

Main Results:

  • Colchicine dose adjustment is indicated for FMF patients with >4 attacks/year.
  • IL1 inhibitors are recommended for FMF patients with persistent attacks (≥6/year) on maximum colchicine doses.
  • Routine genetic testing for MEFV mutations in asymptomatic siblings is not recommended.

Conclusions:

  • This consensus provides a framework for addressing practical FMF management challenges.
  • The recommendations aim to improve daily care for FMF patients.
  • This represents a significant step towards standardizing FMF treatment protocols.
Keywords:
Biological agentsColchicineColchicine resistanceConsensus development conferenceFamilial Mediterranean feverGenetic analysisGuidelinesReviewSiblingsTherapy

Related Concept Videos

Myocarditis III: Medical Management01:14

Myocarditis III: Medical Management

Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...
Rheumatic Heart Disease III: Medical Management01:21

Rheumatic Heart Disease III: Medical Management

Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...
Pericarditis III: Medical Management01:17

Pericarditis III: Medical Management

The primary objectives of managing pericarditis are to determine the underlying cause, provide effective therapy for treatment and symptom relief, and promptly detect signs and symptoms of cardiac tamponade. The following outlines the essential aspects of medical management for pericarditis:ObjectivesDetermine the Cause: Identifying the underlying cause of pericarditis is crucial for targeted treatment. Causes include viral infections, autoimmune diseases, post-cardiac injury syndrome, and...
Myocarditis IV: Nursing Management01:22

Myocarditis IV: Nursing Management

Myocarditis is an inflammatory condition of the myocardium requiring meticulous nursing management for optimal patient outcomes. Effective management begins with a thorough assessment of the patient's medical history, paying close attention to past infections, autoimmune disorders, travel history, and exposure to toxins or drugs. Recent viral infections and systemic diseases are particularly relevant due to their potential role in triggering myocarditis.Physical Examination and MonitoringThe...
Methods of reducing fever01:22

Methods of reducing fever

The signs and symptoms of fever include hot and dry skin, flushed face, thirst, muscle aches, anorexia, headache, tachycardia, tachypnea, and fatigue. Elevated body temperature is reduced using two methods: pharmacological and nonpharmacological. Proper identification and treatment of the root cause of a fever is of utmost importance.
Pharmacological Methods of Reducing Fever:
Endocarditis III: Medical Management01:18

Endocarditis III: Medical Management

Infective endocarditis management involves a multifaceted approach encompassing infection prevention, lifestyle modifications, pharmacological therapy, and surgical management.Infection Prevention:Hand Hygiene: Thorough handwashing is crucial to prevent the spread of infection. Hand hygiene should be performed regularly, especially before and after using the restroom.Oral Hygiene: Good oral hygiene is essential. It includes brushing teeth immediately after waking up and before bed, flossing...