First-Line Treatment for Familial Mediterranean Fever (FMF)
Colchicine is the first-line treatment for Familial Mediterranean Fever and should be started as soon as a clinical diagnosis is made. 1
Colchicine Dosing Protocol
Colchicine dosing should be based on age and clinical response:
| Age Group | Starting Dose | Maximum Dose |
|---|---|---|
| <5 years | 0.5 mg/day | 1.2 mg/day |
| 5-10 years | 0.5-1.0 mg/day | 1.8 mg/day |
| >10 years and adults | 1.0-1.5 mg/day | 3.0 mg/day |
- Dosing can be administered in single or divided doses, depending on tolerance and compliance 1
- For patients with pre-existing complications (e.g., amyloidosis) or greater disease activity, higher initial doses may be warranted
- The ultimate goal of treatment is to achieve complete control of unprovoked attacks and minimize subclinical inflammation between attacks 1
Monitoring and Dose Adjustment
- If attacks persist or subclinical inflammation continues (elevated acute phase reactants), increase colchicine dose by 0.5 mg/day 1
- Monitor response, toxicity, and compliance every 6 months 1
- Check liver enzymes regularly; if elevated more than twice the upper limit of normal, reduce dose and investigate further 1
- In patients with decreased renal function, monitor for signs of colchicine toxicity and adjust dose accordingly 1
Management During Attacks
- During acute FMF attacks, continue the usual dose of colchicine and add NSAIDs for symptomatic relief 1
- Always consider other possible causes when suspecting an attack 1
Special Considerations
- Colchicine should not be discontinued during conception, pregnancy, or lactation 1
- For chronic arthritis in FMF patients, additional medications such as DMARDs, intra-articular steroid injections, or biologics may be needed 1
- In protracted febrile myalgia, glucocorticoids, NSAIDs, or IL-1 blockade may be treatment options 1
Management of Colchicine Resistance/Intolerance
- Patients not responding to the maximum tolerated dose of colchicine (after confirming compliance) are considered colchicine-resistant 1
- For these patients, biological treatments targeting IL-1 are indicated 1, 2
- IL-1 inhibitors such as anakinra, rilonacept, and canakinumab have shown efficacy in colchicine-resistant FMF 2, 3
- However, even when using biologics, it is recommended to continue colchicine at the maximum tolerated dose to prevent amyloidosis 2
Common Pitfalls and Caveats
Gastrointestinal side effects are common with colchicine but can be managed by:
- Starting with a low dose and gradually increasing
- Dividing the daily dose
- Temporary dose reduction during side effects 4
Colchicine toxicity is a serious complication that should be carefully monitored, especially in patients with renal impairment 1
Drug interactions can increase colchicine levels and toxicity risk; avoid concomitant use with CYP3A4 inhibitors 5
Never abruptly discontinue colchicine as this may trigger FMF attacks and increase the risk of amyloidosis 6