C1349068

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FMF Plan of Management Algorithm

Familial Mediterranean Fever (FMF) is an autoinflammatory disorder, and its management algorithm is designed to control acute attacks, prevent complications (especially amyloidosis), and minimize subclinical inflammation between attacks62.

Key Steps in the FMF Management Algorithm

• Diagnosis Confirmation

  • Clinical criteria and, where available, genetic testing to confirm FMF diagnosis.

• Initial Therapy

  • Colchicine is the first-line treatment for all patients with FMF, regardless of attack frequency or severity62.

    • Start with a standard dose and titrate based on response and tolerance.

    • Goal: Complete control of attacks and normalization of inflammatory markers (e.g., serum amyloid A, CRP)62.

• Monitoring and Treat-to-Target Approach

  • Regular assessment of:

    • Frequency and severity of attacks

    • Laboratory markers of inflammation (e.g., CRP, ESR, serum amyloid A)

    • Adherence and side effects62

  • Adjust colchicine dose as needed to achieve target criteria (no attacks, normal inflammatory markers)26.

• Management of Colchicine Resistance or Intolerance

  • If attacks or inflammation persist despite maximum tolerated colchicine:

    • Confirm compliance and exclude other causes.

    • Consider adding or switching to biologic agents (e.g., anti-IL-1 therapies such as anakinra or canakinumab)62.

• Prevention and Monitoring for Complications

  • Regular screening for proteinuria and renal function to detect amyloidosis early.

  • Monitor for colchicine toxicity (especially in patients with renal impairment).

• Patient Education and Support

  • Educate patients and families about the importance of lifelong therapy and adherence.

  • Provide genetic counseling when appropriate.

Consensus and Treat-to-Target Strategies

Recent consensus guidelines emphasize a treat-to-target strategy, with treatment goals including:

• Complete control of unprovoked attacks

• Minimization of subclinical inflammation between attacks

• Prevention of amyloidosis26

A set of target criteria has been developed and approved by expert groups, forming the foundation of this approach2.

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Summary Table: FMF Management Algorithm

Step	Action
Diagnosis	Clinical and genetic confirmation
Initial Therapy	Start colchicine, titrate to effect
Monitoring	Assess attacks, labs, adherence, side effects
Treat-to-Target	Adjust therapy to achieve no attacks, normal inflammation
Colchicine Resistance/Intolerance	Assess compliance, consider biologics (e.g., anti-IL-1)
Complication Prevention	Screen for amyloidosis, monitor renal function
Patient Education	Stress adherence, provide genetic counseling

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This algorithm is based on current international guidelines and recent consensus statements, ensuring transparent, evidence-based treatment decisions for FMF26.

1. https://fetalmedicine.org/research/manage/sga
2. https://pmc.ncbi.nlm.nih.gov/articles/PMC10521475/
3. https://fetalmedicine.org/research/assess/preeclampsia
4. https://courses.fetalmedicine.com/software/Program%20manual-%20Version%202013-280_CE.pdf
5. https://pmc.ncbi.nlm.nih.gov/articles/PMC10530459/
6. https://emedicine.medscape.com/article/330284-guidelines