Familial Mediterranean Fever Guidelines

Updated: Dec 14, 2021
  • Author: John O Meyerhoff, MD; Chief Editor: Herbert S Diamond, MD  more...
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Guidelines Summary

Guidelines from the European League Against Rheumatism (EULAR) for the management of familial Mediterranean fever (FMF) include the following recommendations [23] :

  • Ideally, FMF should be diagnosed and initially treated by a physician with experience in FMF; after diagnosis and initiation of therapy, patients can also be followed by their primary care physician in conjunction with the referral center, but if possible, patientsshould be seen by a physician with experience of FMF at least once per year in the long term.
  • The ultimate goal of treatment in FMF is to obtain complete control of unprovoked attacks and minimize subclinical inflammation between attacks.
  • Treatment with colchicine should be started as soon as a clinical diagnosis is made.
  • Colchicine can be given in single or divided doses, depending on tolerance and compliance.
  • Persistent attacks or subclinical inflammation represent an indication to increase the colchicine dose.
  • Compliant patients who continue to have one or more attacks each month despite receiving the maximally tolerated dose of colchicine for at least 6 months can be considered nonresponders or resistant; alternative biological treatments are indicated in these patients; colchicine should be coadministered with biological therapies, as it may reduce the risk of amyloidosis despite persistence of attacks
  • FMF treatment needs to be intensified in AA amyloidosis, using the maximal tolerated dose of colchicine and supplemented with biologics as required.
  • Periods of physical or emotional stress can trigger FMF attacks, and it may be worth temporarily increasing the dose of colchicine.
  • Response, toxicity, and compliance should be monitored every 6 months.
  • Liver enzymes should be monitored regularly in patients with FMF treated with colchicine; if elevations greater than twofold the upper limit of normal occur, the colchicine dose should be reduced and the cause further investigated.
  • In patients with decreased renal function, the risk of colchicine toxicity is very high, so evidence of toxicity should routinely be sought and if found, the colchicine dose should be reduced accordingly.
  • Colchicine toxicity is a serious complication that should be given adequate consideration and be prevented; potential causes include exceeding the recommended dose (maximum recommended oral doses for treatment of FMF are 3 mg daily in adults and 2 mg daily in children), liver or renal failure, and concomitant administration of other drugs metabolized by cytochrome 3A4.
  • If an attack is suspected, always consider other possible causes; during attacks, continue the usual dose of colchicine and use nonsteroidal anti inflammatory drugs (NSAIDs).
  • Colchicine should not be discontinued during conception, pregnancy, or lactation; current evidence does not justify amniocentesis.
  • In general, men need not stop colchicine prior to conception; in the rare case of azoospermia or oligospermia proven to be related to colchicine, temporary dose reduction or discontinuation may be required.
  • Chronic arthritis in a patient with FMF might need additional medications, such as disease-modifying antirheumatic drugs (DMARDs), intra-articular steroid injections, or biologics.
  • In protracted febrile myalgia, glucocorticoids lead to the resolution of symptoms; NSAID and interleukin-1 blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain.
  • If a patient is stable, with no attacks for more than 5 years and no elevation in acute phase reactants (eg, serum amyloid A protein, C-reactive protein), dose reduction could be considered after expert consultation and with continued monitoring.