eMedicine Specialties > Rheumatology > Miscellaneous Inflammatory Arthritis

Mediterranean Fever, Familial: Follow-up

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
Contributor Information and Disclosures

Updated: Nov 21, 2008

Follow-up

Further Outpatient Care

  • Patients with familial Mediterranean fever (FMF) should be seen regularly to ensure compliance with therapy. Only 2% of 906 patients who were at high risk and compliant developed amyloidosis, compared with 49% of 54 patients who admitted noncompliance.
  • Teenagers are typically a noncompliant group and need long-term daily therapy to prevent chronic complications. For many of these patients, noncompliance is associated with severe symptoms, which may reinforce the need for therapy. Communicating with patients' pharmacies to determine how often they are obtaining refills may be the best way to assess compliance.
  • Perform a urinalysis at every visit, particularly in patients at risk of developing amyloidosis. If proteinuria is present, assess patients carefully for compliance. Exclude other causes of proteinuria (eg, heavy sports activity). If proteinuria is confirmed, increase the daily dose of colchicine.
  • For unknown reasons, hematuria occurs in approximately 5% of patients. Its presence, along with prolonged abdominal or muscle pain, suggests the development of polyarteritis nodosa.

Complications

  • Patients with amyloidosis may develop an acute onset of renal failure if they are stressed by dehydration, infection, or both.
  • Renal vein thrombosis may occur in nephrotic patients. This condition may manifest as abdominal or flank pain, increasing proteinuria, and worsening renal function. Acute anticoagulation may stabilize or improve renal function.

Prognosis

  • Patients who are compliant with daily colchicine can probably expect to have a normal lifespan if colchicine is started before proteinuria develops.
  • Even with amyloidosis, the use of colchicine, dialysis, and renal transplantation should extend a patient's life beyond age 50 years.

Patient Education

  • Patients with FMF need to understand the importance of strict compliance with daily colchicine therapy.

Miscellaneous

Medicolegal Pitfalls

  • In patients with the appropriate ethnic background who present with what appears to be appendicitis or peritonitis, seek a family history of familial Mediterranean fever (FMF), unexplained abdominal pain, or an unnecessary appendectomy (ie, healthy appendix before surgery).
 


More on Mediterranean Fever, Familial

Overview: Mediterranean Fever, Familial
Differential Diagnoses & Workup: Mediterranean Fever, Familial
Treatment & Medication: Mediterranean Fever, Familial
Follow-up: Mediterranean Fever, Familial
References
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References

  1. 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].

  2. 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].

  3. 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].

  4. Lidar M, Livneh A. Familial Mediterranean fever: clinical, molecular and management advancements. Neth J Med. Oct 2007;65(9):318-24. [Medline]. [Full Text].

  5. 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].

  6. 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].

  7. 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].

  8. 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].

  9. Tweezer-Zaks N, Rabinovich E, Lidar M, et al. Interferon-alpha as a treatment modality for colchicine- resistant familial Mediterranean fever. J Rheumatol. Jul 2008;35(7):1362-5. [Medline].

  10. 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].

  11. 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].

  12. Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet. Feb 28 1998;351(9103):659-64. [Medline].

  13. Lidar M, Kedem R, Mor A, et al. Arthritis as the sole episodic manifestation of familial Mediterranean fever. J Rheumatol. May 2005;32(5):859-62. [Medline].

  14. Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum. Oct 1997;40(10):1879-85. [Medline].

  15. Livneh A, Langevitz P, Zemer D, et al. The changing face of familial Mediterranean fever. Semin Arthritis Rheum. Dec 1996;26:612-27. [Medline].

  16. Majeed HA, El-Khateeb M, El-Shanti H, et al. The spectrum of familial Mediterranean fever gene mutations in Arabs: report of a large series. Semin Arthritis Rheum. Jun 2005;34(6):813-8. [Medline].

  17. 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].

  18. Samuels J, Aksentijevich I, Torosyan Y, et al. Familial Mediterranean fever at the millennium. Clinical spectrum, ancient mutations, and a survey of 100 American referrals to the National Institutes of Health. Medicine (Baltimore). Jul 1998;77:268-97. [Medline].

  19. Sohar E, Gafni J, Pras M, et al. Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med. Aug 1967;43(2):227-53. [Medline].

  20. Tunca M, Akar S, Onen F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). Jan 2005;84(1):1-11. [Medline].

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Further Reading

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Keywords

familial Mediterranean fever, FMF, recurrent polyserositis, periodic fever syndrome, amyloidosis, secondary amyloidosis, colchicine therapy, colchicine, appendicitis, peritonitis, amyloid nephropathy, MEFV gene, pyrin, marenostrin, amyloid A, serum amyloid A, nephrotic syndrome, paroxysm, renal vein thrombosis, proteinuria, vasculitis, arthritis, myalgia syndrome, peritonitis, pleuritis, peritoneum, seronegative spondyloarthropathy, chemotactic factor

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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: Pfizer  Honoraria Speaking and teaching

Medical Editor

Carlos J Lozada, MD, Director of Rheumatology Fellowship Program, Associate Professor, Department of Medicine, Division of Rheumatology and Immunology, Jackson Memorial Medical Center, University of Miami 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: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

 
 
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