Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute attack

1st line – analgesic or NSAID + colchicine

Back
ACUTE
|
acute attack
1st line – 

analgesic or NSAID + colchicine

Acute attacks are treated with analgesics or non-steroidal anti-inflammatory drugs (NSAIDs) for symptom relief.

Colchicine should be continued, but a transient increase in its dose should be discouraged due to its inconsistent effectiveness and an increased risk of side effects.[92] When increased doses of colchicine are used to treat an FMF attack, the following considerations are mandatory: increase the doses only during the prodrome of the attack; never exceed the maximum daily dose; monitor side effects; return to the patient's 'normal' dose immediately after the attack.

In severe attacks with significant pain manifestations, stronger analgesics (e.g., oxycodone) may be considered.

Primary options

paracetamol: children: 10-15 mg/kg orally every 4-6 hours when required, maximum 75 mg/kg/day; adults: 500-1000 mg every 4-6 hours when required, maximum 4000 mg/day

or

ibuprofen: children: 10 mg/kg orally every 6-8 hours when required, maximum 30 mg/kg/day; adults: 300-400 mg orally every 6-8 hours when required, maximum 2400 mg/day

or

naproxen: children >2 years of age: 5 mg/kg orally twice daily when required, maximum 1000 mg/day; adults: 500 mg orally twice daily when required, maximum 1250 mg/day

-- AND --

colchicine: children 4-6 years of age: 0.3 to 1.8 mg/day orally given once daily or in 2 divided doses; children 6-12 years of age: 0.9 to 1.8 mg/day orally given once daily or in 2 divided doses; adults: 1.2 to 2.4 mg/day orally given once daily or in 2 divided doses

Secondary options

oxycodone: children: consult specialist for guidance on dose; adults: 5-15 mg orally (immediate-release) every 4-6 hours when required for pain initially, then titrate dose carefully to achieve pain relief

and

colchicine: children 4-6 years of age: 0.3 to 1.8 mg/day orally given once daily or in 2 divided doses; children 6-12 years of age: 0.9 to 1.8 mg/day orally given once daily or in 2 divided doses; adults: 1.2 to 2.4 mg/day orally given once daily or in 2 divided doses

ONGOING

confirmed FMF

1st line – colchicine

Back
ONGOING
|
confirmed FMF
1st line – 

colchicine

The only established treatment for FMF. It is almost 95% effective.[90] It suppresses attacks, prevents amyloidosis, and stabilises proteinuria in patients with amyloid nephropathy.[91]

Patients require lifelong daily treatment. Initially, adherence might be poor due to adverse effects.[104] If poor tolerance (e.g., diarrhoea or abdominal pain) develops, it is recommended that the daily dose be divided into two doses, although some patients do find once-daily dosing more tolerable.[105]

Dose is adjusted based on response, levels of ESR, serum amyloid A, CRP, and fibrinogen.[102][106][107]

Primary options

colchicine: children 4-6 years of age: 0.3 to 1.8 mg/day orally given once daily or in 2 divided doses; children 6-12 years of age: 0.9 to 1.8 mg/day orally given once daily or in 2 divided doses; adults: 1.2 to 2.4 mg/day orally given once daily or in 2 divided doses

Consider – biological agents

Back
ONGOING
|
confirmed FMF
Consider – 

biological agents

Additional treatment recommended for SOME patients in selected patient group

If there is no doubt about the diagnosis and there is poor response, or poor tolerance to colchicine, biological agents (e.g., TNF-alpha antagonist or interleukin [IL]-1 receptor antagonist) may be added to the treatment regimen.[108]

Currently, evidence supporting the use of biological agents is limited. Functional studies suggest that IL-1 is implicated in the inflammatory reaction in FMF; therefore, IL-1 inhibitors are thought to be a good approach in resistant FMF. Studies have confirmed the good response of colchicine-resistant FMF patients to IL-1 inhibitors.[97][98][99] One Cochrane review concluded that anakinra and canakinumab are probably effective, but more research is needed for rilonacept.[100] IL-1 inhibitors are now tried first for colchicine-resistant and/or intolerant patients.[101] Even if an increasing number of IL-1 targeting drugs are currently available, treatment should start with a short half-life drug (i.e., anakinra) in order to test effectiveness. Medium or long half-life drugs (i.e., rilonacept, canakinumab) should be considered only if the short half-life drug is effective.[99][102] Canakinumab and anakinra are approved for FMF in some countries.

TNF-alpha antagonists (e.g., etanercept, infliximab) may be prescribed in patients with important articular involvement or may be tried second line.[102][103]

Primary options

canakinumab: children and adults body weight ≤40 kg: 2 mg/kg subcutaneously every 4 weeks initially, increase to 4 mg/kg every 4 weeks if clinical response is inadequate; adults or body weight >40 kg: 150 mg subcutaneously every 4 weeks initially, increase to 300 mg every 4 weeks if clinical response is inadequate

OR

anakinra: children and adults body weight ≤50 kg: 1-2 mg/kg subcutaneously once daily; adults or body weight >50 kg: 100 mg subcutaneously once daily

OR

rilonacept: children and adults: consult specialist for guidance on dose

Secondary options

etanercept: children and adults: consult specialist for guidance on dose

OR

infliximab: children and adults: consult specialist for guidance on dose

Consider – oral corticosteroid

Back
ONGOING
|
confirmed FMF
Consider – 

oral corticosteroid

Additional treatment recommended for SOME patients in selected patient group

May be required for protracted myalgia for as long as 6 weeks.

Primary options

prednisolone: children and adults: 1 mg/kg/day orally

Consider – NSAID

Back
ONGOING
|
confirmed FMF
Consider – 

NSAID

Additional treatment recommended for SOME patients in selected patient group

May be required for protracted myalgia for as long as 6 weeks.

Primary options

ibuprofen: children: 10 mg/kg orally every 6-8 hours when required, maximum 30 mg/kg/day; adults: 300-400 mg orally every 6-8 hours when required, maximum 2400 mg/day

OR

naproxen: children >2 years of age: 5 mg/kg orally twice daily when required, maximum 1000 mg/day; adults: 500 mg orally twice daily when required, maximum 1250 mg/day

back arrow

Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

Use of this content is subject to our disclaimer