Familial mediterranean fever medical therapy: Difference between revisions
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==Medical Therapy== | ==Medical Therapy== | ||
*Pharmacologic medical therapy of choice for familial Mediterranean fever is colchicine. | *Pharmacologic medical therapy of choice for familial Mediterranean fever is colchicine. | ||
'''Colchicine''' | '''Colchicine''' | ||
*This drug is critical for the treatment of FMF. It has been indicated to have a protective effect against amyloidosis.<ref name="OzenDemirkaya2016">{{cite journal|last1=Ozen|first1=Seza|last2=Demirkaya|first2=Erkan|last3=Erer|first3=Burak|last4=Livneh|first4=Avi|last5=Ben-Chetrit|first5=Eldad|last6=Giancane|first6=Gabriella|last7=Ozdogan|first7=Huri|last8=Abu|first8=Illana|last9=Gattorno|first9=Marco|last10=Hawkins|first10=Philip N|last11=Yuce|first11=Sezin|last12=Kallinich|first12=Tilmann|last13=Bilginer|first13=Yelda|last14=Kastner|first14=Daniel|last15=Carmona|first15=Loreto|title=EULAR recommendations for the management of familial Mediterranean fever|journal=Annals of the Rheumatic Diseases|volume=75|issue=4|year=2016|pages=644–651|issn=0003-4967|doi=10.1136/annrheumdis-2015-208690}}</ref> | *This drug is critical for the treatment of FMF. It has been indicated to have a protective effect against amyloidosis.<ref name="OzenDemirkaya2016">{{cite journal|last1=Ozen|first1=Seza|last2=Demirkaya|first2=Erkan|last3=Erer|first3=Burak|last4=Livneh|first4=Avi|last5=Ben-Chetrit|first5=Eldad|last6=Giancane|first6=Gabriella|last7=Ozdogan|first7=Huri|last8=Abu|first8=Illana|last9=Gattorno|first9=Marco|last10=Hawkins|first10=Philip N|last11=Yuce|first11=Sezin|last12=Kallinich|first12=Tilmann|last13=Bilginer|first13=Yelda|last14=Kastner|first14=Daniel|last15=Carmona|first15=Loreto|title=EULAR recommendations for the management of familial Mediterranean fever|journal=Annals of the Rheumatic Diseases|volume=75|issue=4|year=2016|pages=644–651|issn=0003-4967|doi=10.1136/annrheumdis-2015-208690}}</ref> | ||
'''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) | |||
'''Parenteral colchicine''' | |||
*Colchicine may be administered intravenously every week in critically ill patients, but this method is associated with increased risk of toxicity.<ref name="pmid19796546">{{cite journal |vauthors=Rozenbaum M, Boulman N, Feld J, Avshovich N, Petrovich S, Elias M, Slobodin G, Rosner I |title=Intravenous colchicine treatment for six months: adjunctive therapy in familial Mediterranean fever (FMF) unresponsive to oral colchicine |journal=Clin. Exp. Rheumatol. |volume=27 |issue=2 Suppl 53 |pages=S105 |date=2009 |pmid=19796546 |doi= |url=}}</ref><ref name="pmid14719203">{{cite journal |vauthors=Lidar M, Kedem R, Langevitz P, Pras M, Livneh A |title=Intravenous colchicine for treatment of patients with familial Mediterranean fever unresponsive to oral colchicine |journal=J. Rheumatol. |volume=30 |issue=12 |pages=2620–3 |date=December 2003 |pmid=14719203 |doi= |url=}}</ref> | |||
'''Specific instructions'''<ref name="pmid26802180">{{cite journal |vauthors=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 |title=EULAR recommendations for the management of familial Mediterranean fever |journal=Ann. Rheum. Dis. |volume=75 |issue=4 |pages=644–51 |date=April 2016 |pmid=26802180 |doi=10.1136/annrheumdis-2015-208690 |url=}}</ref> | '''Specific instructions'''<ref name="pmid26802180">{{cite journal |vauthors=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 |title=EULAR recommendations for the management of familial Mediterranean fever |journal=Ann. Rheum. Dis. |volume=75 |issue=4 |pages=644–51 |date=April 2016 |pmid=26802180 |doi=10.1136/annrheumdis-2015-208690 |url=}}</ref> | ||
*It is generally recommended to give the dose once daily. However, it may be divided in case of side effects. | *It is generally recommended to give the dose once daily. However, it may be divided in case of side effects. | ||
| Line 33: | Line 32: | ||
*The persistence of attacks or subclinical inflammation is an indication to increase the dose of colchicine. | *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. | *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. | *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. | *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. | *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. | *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. | *Dose reduction may be considered in a patient with no attack for more than 5 years and no elevation in acute phase reactant. | ||
| Line 41: | Line 41: | ||
*Gastrointestinal side effects <ref name="pmid1788551">{{cite journal |vauthors=Putterman C, Ben-Chetrit E, Caraco Y, Levy M |title=Colchicine intoxication: clinical pharmacology, risk factors, features, and management |journal=Semin. Arthritis Rheum. |volume=21 |issue=3 |pages=143–55 |date=December 1991 |pmid=1788551 |doi= |url=}}</ref><ref name="pmid9726336">{{cite journal |vauthors=Ben-Chetrit E, Levy M |title=Colchicine: 1998 update |journal=Semin. Arthritis Rheum. |volume=28 |issue=1 |pages=48–59 |date=August 1998 |pmid=9726336 |doi= |url=}}</ref> | *Gastrointestinal side effects <ref name="pmid1788551">{{cite journal |vauthors=Putterman C, Ben-Chetrit E, Caraco Y, Levy M |title=Colchicine intoxication: clinical pharmacology, risk factors, features, and management |journal=Semin. Arthritis Rheum. |volume=21 |issue=3 |pages=143–55 |date=December 1991 |pmid=1788551 |doi= |url=}}</ref><ref name="pmid9726336">{{cite journal |vauthors=Ben-Chetrit E, Levy M |title=Colchicine: 1998 update |journal=Semin. Arthritis Rheum. |volume=28 |issue=1 |pages=48–59 |date=August 1998 |pmid=9726336 |doi= |url=}}</ref> | ||
*Abdominal cramps | *Abdominal cramps | ||
* | *Hyperperistalsis | ||
*Diarrhoea | |||
*Vomiting | |||
*In case of colchicine resistance, other options include: | |||
**NSAIDs | |||
**IL-1 blockade agents | |||
==NSAIDS== | ==NSAIDS== | ||
Indications: | Indications: | ||
Revision as of 15:31, 29 May 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Clinical practice guidelines direct treatment[1]: 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.
Colchicine
- This drug is critical for the treatment of FMF. It has been indicated to have a protective effect against amyloidosis.[2]
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)
Parenteral colchicine
- Colchicine may be administered intravenously every week in critically ill patients, but this method is associated with increased risk of toxicity.[3][4]
Specific instructions[1]
- 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 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
- Gastrointestinal side effects [5][6]
- Abdominal cramps
- Hyperperistalsis
- Diarrhoea
- Vomiting
- In case of colchicine resistance, other options include:
- NSAIDs
- IL-1 blockade agents
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:[2]
- 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.[7]
- 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.[8] 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.[9] Average age was and 24 years and 7% of subjects were homozygous for M694V This trial was published and registered (NCT00582907).
References
- ↑ 1.0 1.1 Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G; et al. (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.
- ↑ 2.0 2.1 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Ben-Chetrit E, Levy M (August 1998). "Colchicine: 1998 update". Semin. Arthritis Rheum. 28 (1): 48–59. PMID 9726336.
- ↑ 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.
- ↑ 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.
- ↑ 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.