Familial mediterranean fever medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Clinical practice guidelines direct treatment: The mainstay of treatment for familial mediterranean fever is medical therapy. 03. "Treatment with colchicine should start as soon as a clinical diagnosis is made"

17. "In protracted febrile myalgia, glucocorticoids lead to the resolution of symptoms; NSAID and IL-1-blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain"

Medical Therapy

• Pharmacologic medical therapy of choice for familial Mediterranean fever is colchicine.
• In case of colchicine resistance, other options include:
  • NSAIDs
  • IL-1 blockade agents

Colchicine

• This drug is critical for the treatment of FMF. It has been indicated to have a protective effect against amyloidosis.
• Adult
  • 1.2-1.8 mg PO daily (maximum, 3 mg per day)
  • Higher initial doses (up to 2 mg per day) may be indicated in those with:
    • Preexisting complications (eg, renal amyloidosis)
    • High frequency of attacks
    • Longer duration of each attack
    • Joint involvement

Specific instructions

• It is generally recommended to give the dose once daily. However, it may be divided in case of side effects.
• After initiation of therapy, the patient should be evaluated closely for 3 to 6 months to assess its efficacy on attack severity and frequency.
• Treatment preferably should be initiated with a low dose and then the dose be increased according to the patients' response.
• CRP, or SAA, or both should be monitored at least every three months during dosage increase to determine the necessary dose.
• In case of preexisting severe renal insufficiency (GFR< 10 ml/min), colchicine dosage should be decreased to 50% due to the potential renal toxicity.
• Pediatric
  • Children younger than 5 years of age, =< 0.5 mg per day (maximum, 0.6 mg per day)
  • Children aged 5 to 10 years of age, 0.5 to 1 mg per day (maximum, 1.2 mg per day)
  • Children older than 10 years of age, 1 to 1.5 mg per day (maximum, 2 mg per day)

Side effects

• Gastrointestinal side effects

IL-1-blockade

• Indications:
  • Compliant patients who continue to have ≥ 1 attacks each month despite receiving the maximally tolerated dose for at least 6 months are considered to be a non-responder or

resistant to colchicine therapy; alternative biological treatments are indicated.

A systematic review of IL-1 blockade with anakinra, canakinumab, and rilonacept found only one randomized controlled trial.

• anakinra: benefit in one small, randomized controlled trial in which the median number of attacks per month dropped from 3.5 with placebo to 1.7 with anakinra. Average age was 37 years and 83% of subjects were homozygous for M694V. This trial was published and registered (NCT01705756).
• canakinumab: no randomized controlled trials.
• rilonacept: benefit in one small, randomized controlled trial in which the median number of attacks per month dropped from 2 with placebo to 0.77 with rilonacept. Average age was and 24 years and 7% of subjects were homozygous for M694V This trial was published and registered (NCT00582907).