Familial mediterranean fever medical therapy

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Template:Familial Mediterranean fever Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overview

The mainstay of treatment for familial Mediterranean fever is medical therapy with colchicine.

Medical Therapy

Colchicine

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:

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)

Parenteral colchicine

  • Colchicine may be administered intravenously every week in critically ill patients, but this method is associated with increased risk of toxicity.[2][3]

Specific instructions

  • It is generally recommended to give the dose once daily. However, it may be divided in case of side effects.[4]
  • 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 sub clinical 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 decreased renal function, symptoms and signs of colchicine toxicity as well as CPK level should be monitored regularly to reduce colchicine dose accordingly.
  • 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

In case of colchicine resistance, other options include:[7][8]

  • IL-1 blockade agents

NSAIDS

Indications:[4]

Glucocorticoids

  • Theses medications may shorten the duration of attack, but may also increase the frequency of attacks.[9]

Indications:

Disease modifying anti rheumatic drugs (DMARDs)

Indications:[11]

  • Chronic arthritis refractory to treatment with colchicine

IL-1-blockade

  • Indications:[1]
    • 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.[12]

  • 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.[13] 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.[14] Average age was and 24 years and 7% of subjects were homozygous for M694V This trial was published and registered (NCT00582907).

References

  1. ↑ 1.0 1.1 Ozen, Seza; Demirkaya, Erkan; Erer, Burak; Livneh, Avi; Ben-Chetrit, Eldad; Giancane, Gabriella; Ozdogan, Huri; Abu, Illana; Gattorno, Marco; Hawkins, Philip N; Yuce, Sezin; Kallinich, Tilmann; Bilginer, Yelda; Kastner, Daniel; Carmona, Loreto (2016). "EULAR recommendations for the management of familial Mediterranean fever". Annals of the Rheumatic Diseases. 75 (4): 644–651. doi:10.1136/annrheumdis-2015-208690. ISSN 0003-4967.
  2. ↑ Rozenbaum M, Boulman N, Feld J, Avshovich N, Petrovich S, Elias M, Slobodin G, Rosner I (2009). "Intravenous colchicine treatment for six months: adjunctive therapy in familial Mediterranean fever (FMF) unresponsive to oral colchicine". Clin. Exp. Rheumatol. 27 (2 Suppl 53): S105. PMID 19796546.
  3. ↑ Lidar M, Kedem R, Langevitz P, Pras M, Livneh A (December 2003). "Intravenous colchicine for treatment of patients with familial Mediterranean fever unresponsive to oral colchicine". J. Rheumatol. 30 (12): 2620–3. PMID 14719203.
  4. ↑ 4.0 4.1 Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G, Ozdogan H, Abu I, Gattorno M, Hawkins PN, Yuce S, Kallinich T, Bilginer Y, Kastner D, Carmona L (April 2016). "EULAR recommendations for the management of familial Mediterranean fever". Ann. Rheum. Dis. 75 (4): 644–51. doi:10.1136/annrheumdis-2015-208690. PMID 26802180.
  5. ↑ Putterman C, Ben-Chetrit E, Caraco Y, Levy M (December 1991). "Colchicine intoxication: clinical pharmacology, risk factors, features, and management". Semin. Arthritis Rheum. 21 (3): 143–55. PMID 1788551.
  6. ↑ Ben-Chetrit E, Levy M (August 1998). "Colchicine: 1998 update". Semin. Arthritis Rheum. 28 (1): 48–59. PMID 9726336.
  7. ↑ Roldan, Rosa; Ruiz, Adela M.; Miranda, Maria D.; Collantes, Eduardo (2008). "Anakinra: New therapeutic approach in children with Familial Mediterranean Fever resistant to colchicine". Joint Bone Spine. 75 (4): 504–505. doi:10.1016/j.jbspin.2008.04.001. ISSN 1297-319X.
  8. ↑ Ben-Zvi I, Livneh A (May 2014). "Colchicine failure in familial Mediterranean fever and potential alternatives: embarking on the anakinra trial". Isr. Med. Assoc. J. 16 (5): 271–3. PMID 24979828.
  9. ↑ Amital H, Ben-Chetrit E (2004). "Therapeutic approaches to familial Mediterranean fever. What do we know and where are we going to?". Clin. Exp. Rheumatol. 22 (4 Suppl 34): S4–7. PMID 15515775.
  10. ↑ Kaplan E, Mukamel M, Barash J, Brik R, Padeh S, Berkun Y, Uziel Y, Tauber T, Amir J, Harel L (2007). "Protracted febrile myalgia in children and young adults with familial Mediterranean fever: analysis of 15 patients and suggested criteria for working diagnosis". Clin. Exp. Rheumatol. 25 (4 Suppl 45): S114–7. PMID 17949564.
  11. ↑ Demirag, Mehmet D.; Ozturk, Mehmet A.; Goker, Berna; Haznedaroglu, Seminur (2008). "Intramuscular gold for the treatment of seronegative spondyloarthropathy associated with familial Mediterranean fever". Rheumatology International. 29 (1): 77–79. doi:10.1007/s00296-008-0631-7. ISSN 0172-8172.
  12. ↑ van der Hilst JCh, Moutschen M, Messiaen PE, Lauwerys BR, Vanderschueren S (2016). "Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature". Biologics. 10: 75–80. doi:10.2147/BTT.S102954. PMC 4831592. PMID 27110096.
  13. ↑ Ben-Zvi I, Kukuy O, Giat E, Pras E, Feld O, Kivity S; et al. (2017). "Anakinra for Colchicine-Resistant Familial Mediterranean Fever: A Randomized, Double-Blind, Placebo-Controlled Trial". Arthritis Rheumatol. 69 (4): 854–862. doi:10.1002/art.39995. PMID 27860460.
  14. ↑ Hashkes PJ, Spalding SJ, Giannini EH, Huang B, Johnson A, Park G; et al. (2012). "Rilonacept for colchicine-resistant or -intolerant familial Mediterranean fever: a randomized trial". Ann Intern Med. 157 (8): 533–41. doi:10.7326/0003-4819-157-8-201210160-00003. PMID 23070486.
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