Familial mediterranean fever medical therapy

Familial Mediterranean Fever Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Familial Mediterranean Fever from other Diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
Diagnostic study of choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X ray
Echocardiography and Ultrasound
CT scan
MRI
Other imaging Studies
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary prevention
Secondary prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Familial mediterranean fever medical therapy On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Familial mediterranean fever medical therapy All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Familial mediterranean fever medical therapy
CDC on Familial mediterranean fever medical therapy
Familial mediterranean fever medical therapy in the news
Blogs on Familial mediterranean fever medical therapy
Directions to Hospitals Treating Familial mediterranean fever
Risk calculators and risk factors for Familial mediterranean fever medical therapy

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
• 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)

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.
• The persistence of attacks or subclinical inflammation is an indication to increase the dose of colchicine.
• Emotional or physical stress can trigger FMF attacks. Temporary increase in colchicine dose may be considered during stress.
• During medical therapy with colchicine, liver function test should be monitored regularly; any elevation greater than twofold the upper limit of normal is an indication for dose reduction.
• CRP, or SAA, or both should be monitored at least every three months during dosage increase to determine the necessary dose.
• Colchicine may be continued during conception, pregnancy, and lactation.
• In case of preexisting severe renal insufficiency (GFR< 10 ml/min), colchicine dosage should be decreased to 50% due to the potential renal toxicity.
• Dose reduction may be considered in a patient with no attack for more than 5 years and no elevation in acute phase reactant.

Side effects

• Gastrointestinal side effects
• Abdominal cramps

NSAIDS

Indications:

• Exertional leg pain

Glucocorticoids

Indications:

• Protracted febrile myalgia which is severe disabling myalgia with at least 5 days duration in a patient with FMF.

Disease modifying antirheumatic drugs (DMARDs)

Indications:

• Chronic arthritis

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).