Familial mediterranean fever medical therapy: Difference between revisions
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{{Familial mediterranean fever}} | {{Familial mediterranean fever}} | ||
{{CMG}} | {{CMG}} | ||
==Overview== | ==Overview== | ||
[[Clinical practice guideline]]s direct treatment<ref name="pmid26802180">{{cite journal| author=Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G et al.| title=EULAR recommendations for the management of familial Mediterranean fever. | journal=Ann Rheum Dis | year= 2016 | volume= 75 | issue= 4 | pages= 644-51 | pmid=26802180 | doi=10.1136/annrheumdis-2015-208690 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26802180 }} </ref>: | [[Clinical practice guideline]]s direct treatment<ref name="pmid26802180">{{cite journal| author=Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G et al.| title=EULAR recommendations for the management of familial Mediterranean fever. | journal=Ann Rheum Dis | year= 2016 | volume= 75 | issue= 4 | pages= 644-51 | pmid=26802180 | doi=10.1136/annrheumdis-2015-208690 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26802180 }} </ref>: | ||
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" | 03. "Treatment with [[colchicine]] should start as soon as a clinical diagnosis is made" | ||
17. "In protracted febrile myalgia, [[glucocorticoid]]s lead to the resolution of symptoms; NSAID and [[Interleukin_1|IL-1]]-blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain" | 17. "In protracted febrile myalgia, [[glucocorticoid]]s lead to the resolution of symptoms; NSAID and [[Interleukin_1|IL-1]]-blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain" | ||
==Medical Therapy== | |||
*Pharmacologic medical therapies for familial mediterranean fever include (either) [therapy 1], [therapy 2], and/or [therapy 3]. | |||
*** 1.1.1 '''Adult''' | |||
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' | |||
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days | |||
*** 1.1.2 '''Pediatric''' | |||
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''') | |||
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) | |||
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day) | |||
==IL-1-blockade== | ==IL-1-blockade== | ||
Revision as of 14:23, 28 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 therapies for familial mediterranean fever include (either) [therapy 1], [therapy 2], and/or [therapy 3].
- 1.1.1 Adult
- 1.1.2 Pediatric
IL-1-blockade
A systematic review of IL-1 blockade with anakinra, canakinumab, and rilonacept found only one randomized controlled trial.[2]
- 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.[3] 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.[4] Average age was and 24 years and 7% of subjects were homozygous for M694V This trial was published and registered (NCT00582907).
References
- ↑ 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.
- ↑ 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.