From the Guidelines
The best management approach for Familial Mediterranean Fever (FMF) is to start treatment with colchicine as soon as a clinical diagnosis is made, with a daily dose of 1.0-1.5 mg in adults and 0.5-1.0 mg in children, which can be increased up to a maximum tolerated dose of 2 mg in children and 3 mg in adults. This approach is based on the EULAR recommendations for the management of FMF, which emphasize the importance of early treatment with colchicine to prevent acute inflammatory attacks and reduce the risk of developing amyloidosis, a serious long-term complication of FMF 1.
Key Considerations
- Colchicine dose can be increased by 0.5 mg/day every 2 weeks until the maximum tolerated dose is reached or until the frequency and severity of attacks are reduced, with monitoring of CRP, SAA protein, or both at least every 3 months 1.
- For patients who cannot tolerate colchicine or have resistant disease, IL-1 inhibitors such as anakinra or canakinumab can be considered as second-line options 1.
- During acute attacks, NSAIDs or short courses of corticosteroids may provide symptomatic relief, and regular monitoring for proteinuria is essential to detect early signs of amyloidosis 1.
- Genetic counseling is also recommended for patients with FMF, particularly those planning to have children, as the disease follows an autosomal recessive inheritance pattern 1.
Monitoring and Follow-up
- Patients should be monitored every 6 months to evaluate the frequency and character of attacks, as well as to monitor APR response in between attacks 1.
- Blood tests should be performed 3 months after dose reduction, and patients must remain under clinical observation and perform periodic laboratory evaluation of SAA protein or CRP and urinary protein 1.
- Dose reduction should be avoided in patients who may be unwilling to comply with these strict constraints, and should only be considered in patients who have been stable with no attacks for several years and have not had elevated APR 1.
From the FDA Drug Label
ILARIS (canakinumab) is an interleukin-1β (IL-1β) blocker indicated for the treatment of the following autoinflammatory Periodic Fever Syndromes: ... Familial Mediterranean Fever (FMF) ILARIS is indicated for the treatment of Familial Mediterranean Fever (FMF) in adult and pediatric patients. The recommended weight-based dosage of ILARIS for patients with ... FMF is: For patients > 40 kg: 150 mg subcutaneously, every 4 weeks The dosage can be increased to 300 mg every 4 weeks if the clinical response is not adequate. For patients ≤ 40 kg: 2 mg/kg administered subcutaneously, every 4 weeks. The dosage can be increased to 4 mg/kg every 4 weeks if the clinical response is not adequate.
The best management approach for Familial Mediterranean Fever (FMF) is the use of canakinumab (ILARIS), with a recommended weight-based dosage of:
- 150 mg subcutaneously every 4 weeks for patients > 40 kg, which can be increased to 300 mg every 4 weeks if the clinical response is not adequate.
- 2 mg/kg subcutaneously every 4 weeks for patients ≤ 40 kg, which can be increased to 4 mg/kg every 4 weeks if the clinical response is not adequate 2.
From the Research
Management Approach for Familial Mediterranean Fever (FMF)
The management of FMF involves the use of colchicine as the primary treatment to prevent attacks and amyloidosis 3. However, some patients may be resistant or intolerant to colchicine, and alternative treatments such as biologic agents may be necessary 4, 5, 6.
Colchicine Treatment
- The minimal daily dose of colchicine in adults is 1.0 mg/day, but the dose in children is not well established 3.
- Colchicine can prevent activation of neutrophils and inhibit the assembly of microtubules and mitotic spindle formation 3.
- Gastrointestinal side effects are common with colchicine treatment, and a lactose-free diet and treatment of intestinal bacterial overgrowth and/or Hp-infection may improve tolerance 3.
Biologic Agents
- Anti-interleukin-1 (anti-IL-1) agents, such as anakinra and canakinumab, have been shown to be effective in patients with colchicine-resistant or -intolerant FMF 4, 5, 6.
- These agents have a better safety profile and fewer drug-drug interactions compared to colchicine 4.
- Canakinumab has been shown to be safe and effective in recipients of kidney transplants with FMF-related amyloidosis 7.