Familial Mediterranean Fever Treatment & Management

  • Author: John O Meyerhoff, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jan 19, 2012
 

Medical Care

Colchicine is so effective in preventing attacks of familial Mediterranean fever (FMF) and preventing the development of amyloidosis that the most important aspects of medical care are to make the correct diagnosis and to institute therapy.

  • Administer colchicine therapy daily (0.6 mg bid or 0.5 mg bid, depending on the dosage form available) in patients at risk of developing amyloidosis (eg, North African Jewish people, Turkish people, Armenian people living in Armenia). Other Sephardic Jewish people and Arabic people are at lower risk but also probably require daily colchicine therapy.
  • Ashkenazi Jewish people and Armenian people living in America seem to be at extremely low risk of amyloidosis and may need treatment only to prevent attacks. If attacks are rare and patients can determine when they are beginning, treatment with intermittent colchicine therapy at the onset of attacks may be sufficient.
  • The regimen for acute attacks in patients not taking daily colchicine is 0.6 mg every hour for 4 doses, then 0.6 mg every 2 hours for 2 doses and then 0.6 mg every 12 hours for 4 doses. Colchicine should be started as soon as the patient recognizes that an attack is occurring. If the initial doses are effective, patients may be able to do without the later doses, but this varies from patient to patient.
  • In patients who do not respond to twice-a-day dosing, administer colchicine 3, or even 4, times a day. In patients who have difficulty tolerating colchicine, start therapy at once-a-day dosing and gradually increase the dose. In patients whose conditions were not responsive to oral colchicine, the addition of 1 mg IV once a week reduced the number of attacks in 10 of 13 patients and the severity of attacks in 6 of 13 patients.[6]
  • Some patients develop lactose intolerance and may respond to a lactose-free diet.
  • In patients whose conditions do not respond to colchicine, the use of interferon alpha, the tumor necrosis factor–blocking drug etanercept,[7] and the IL-1 receptor antagonist anakinra[8] may be effective. Interferon alpha has been used in an intermittent fashion and as prophylaxis, with varying results.[9, 10, 11]
  • Colchicine also stabilizes the amount of proteinuria in patients with amyloid nephropathy. Renal disease may resolve in patients with a creatinine level of less than 1.5 mg/dL who are treated with more than 1.5 mg/d of colchicine.
  • Hemodialysis can be used for patients who develop renal failure. Peritoneal dialysis tends to increase the number of abdominal attacks.
  • Patients who experience episodes of prolonged myalgia with fever and severe pain may need treatment with prednisone (1 mg/kg) for as long as 6 weeks.
  • Patients with exertional lower extremity muscle pain respond to rest.
  • Treat patients with fibromyalgia with the usual agents for this condition.
  • Patients who develop seronegative spondyloarthropathy are treated with nonsteroidal anti-inflammatory drugs. Some of these patients require remission-type drugs (as used in rheumatoid arthritis) and receive follow-up care by a rheumatologist.
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Surgical Care

Before the advent of colchicine therapy, renal transplantation was performed in patients with end-stage renal disease due to amyloid nephropathy. Now, renal failure develops only in patients who are not compliant with therapy or those who cannot tolerate adequate doses of colchicine.

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Consultations

  • Since the advent of colchicine therapy, most treated patients are asymptomatic and do not need consultation with a specialist.
  • Consider consultation with a nephrologist for patients with proteinuria that is not responsive to colchicine.
  • Consultation with a rheumatologist is indicated in patients with the following conditions:
    • Seronegative spondyloarthropathy not responsive to nonsteroidal anti-inflammatory drugs
    • Fibromyalgia not responsive to the usual treatments
    • Coexistent Henoch-Schönlein purpura, polyarteritis nodosa, or Behçet disease
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Contributor Information and Disclosures
Author

John O Meyerhoff, MD  Assistant Professor, Department of Internal Medicine, Johns Hopkins University School of Medicine; Clinical Scholar in Rheumatology, Department of Medicine, Sinai Hospital of Baltimore

John O Meyerhoff, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Carlos J Lozada, MD  Director of Rheumatology Fellowship Program, Professor, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Pfizer Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Elliot Goldberg, MD  Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

References
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  2. Gershoni-Baruch R, Brik R, Shinawi M, Livneh A. The differential contribution of MEFV mutant alleles to the clinical profile of familial Mediterranean fever. Eur J Hum Genet. Feb 2002;10(2):145-9. [Medline]. [Full Text].

  3. Tomiyama N, Higashiuesato Y, Oda T, et al. MEFV mutation analysis of familial Mediterranean fever in Japan. Clin Exp Rheumatol. Jan-Feb 2008;26(1):13-7. [Medline].

  4. Kim S, Ikusaka M, Mikasa G, et al. Clinical study of 7 cases of familial Mediterranean fever with MEFV gene mutation. Intern Med. 2007;46(5):221-5. [Medline]. [Full Text].

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  6. Lidar M, Kedem R, Langevitz P, et al. Intravenous colchicine for treatment of patients with familial Mediterranean fever unresponsive to oral colchicine. J Rheumatol. Dec 2003;30(12):2620-3. [Medline].

  7. Mor A, Pillinger MH, Kishimoto M, et al. Familial Mediterranean fever successfully treated with etanercept. J Clin Rheumatol. Feb 2007;13(1):38-40. [Medline].

  8. Roldan R, Ruiz AM, Miranda MD, et al. Anakinra: new therapeutic approach in children with Familial Mediterranean Fever resistant to colchicine. Joint Bone Spine. Jul 2008;75(4):504-5. [Medline].

  9. Tunca M, Akar S, Soyturk M, et al. The effect of interferon alpha administration on acute attacks of familial Mediterranean fever: A double-blind, placebo-controlled trial. Clin Exp Rheumatol. Jul-Aug 2004;22(4 Suppl 34):S37-40. [Medline].

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  11. Calguneri M, Apras S, Ozbalkan Z, et al. The efficacy of continuous interferon alpha administration as an adjunctive agent to colchicine-resistant familial Mediterranean fever patients. Clin Exp Rheumatol. Jul-Aug 2004;22(4 Suppl 34):S41-4. [Medline].

  12. Ben-Chetrit E, Berkun Y, Ben-Chetrit E, et al. The outcome of pregnancy in the wives of men with familial mediterranean fever treated with colchicine. Semin Arthritis Rheum. Oct 2004;34(2):549-52. [Medline].

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  18. Meyerhoff J. Familial Mediterranean fever: report of a large family, review of the literature, and discussion of the frequency of amyloidosis. Medicine (Baltimore). Jan 1980;59(1):66-77. [Medline].

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