Familial Mediterranean Fever Treatment & Management

Colchicine is so effective in preventing attacks of familial Mediterranean fever (FMF) and preventing the development of amyloidosis that the most important aspects of medical care are to make the correct diagnosis and to institute therapy.

• Administer colchicine therapy daily (0.6 mg bid or 0.5 mg bid, depending on the dosage form available) in patients at risk of developing amyloidosis (eg, North African Jewish people, Turkish people, Armenian people living in Armenia). Other Sephardic Jewish people and Arabic people are at lower risk but also probably require daily colchicine therapy.
• Ashkenazi Jewish people and Armenian people living in America seem to be at extremely low risk of amyloidosis and may need treatment only to prevent attacks. If attacks are rare and patients can determine when they are beginning, treatment with intermittent colchicine therapy at the onset of attacks may be sufficient.
• The regimen for acute attacks in patients not taking daily colchicine is 0.6 mg every hour for 4 doses, then 0.6 mg every 2 hours for 2 doses and then 0.6 mg every 12 hours for 4 doses. Colchicine should be started as soon as the patient recognizes that an attack is occurring. If the initial doses are effective, patients may be able to do without the later doses, but this varies from patient to patient.
• In patients who do not respond to twice-a-day dosing, administer colchicine 3, or even 4, times a day. In patients who have difficulty tolerating colchicine, start therapy at once-a-day dosing and gradually increase the dose. In patients whose conditions were not responsive to oral colchicine, the addition of 1 mg IV once a week reduced the number of attacks in 10 of 13 patients and the severity of attacks in 6 of 13 patients.^[6]
• Some patients develop lactose intolerance and may respond to a lactose-free diet.
• In patients whose conditions do not respond to colchicine, the use of interferon alpha, the tumor necrosis factor–blocking drug etanercept,^[7] and the IL-1 receptor antagonist anakinra^[8] may be effective. Interferon alpha has been used in an intermittent fashion and as prophylaxis, with varying results.^{[9, 10, 11]}
• Colchicine also stabilizes the amount of proteinuria in patients with amyloid nephropathy. Renal disease may resolve in patients with a creatinine level of less than 1.5 mg/dL who are treated with more than 1.5 mg/d of colchicine.
• Hemodialysis can be used for patients who develop renal failure. Peritoneal dialysis tends to increase the number of abdominal attacks.
• Patients who experience episodes of prolonged myalgia with fever and severe pain may need treatment with prednisone (1 mg/kg) for as long as 6 weeks.
• Patients with exertional lower extremity muscle pain respond to rest.
• Treat patients with fibromyalgia with the usual agents for this condition.
• Patients who develop seronegative spondyloarthropathy are treated with nonsteroidal anti-inflammatory drugs. Some of these patients require remission-type drugs (as used in rheumatoid arthritis) and receive follow-up care by a rheumatologist.

Before the advent of colchicine therapy, renal transplantation was performed in patients with end-stage renal disease due to amyloid nephropathy. Now, renal failure develops only in patients who are not compliant with therapy or those who cannot tolerate adequate doses of colchicine.

• Since the advent of colchicine therapy, most treated patients are asymptomatic and do not need consultation with a specialist.
• Consider consultation with a nephrologist for patients with proteinuria that is not responsive to colchicine.
• Consultation with a rheumatologist is indicated in patients with the following conditions:

  • Seronegative spondyloarthropathy not responsive to nonsteroidal anti-inflammatory drugs
  • Fibromyalgia not responsive to the usual treatments
  • Coexistent Henoch-Schönlein purpura, polyarteritis nodosa, or Behçet disease