Familial Mediterranean Fever Follow-up

Updated: Sep 30, 2016
  • Author: John O Meyerhoff, MD; Chief Editor: Herbert S Diamond, MD  more...
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Follow-up

Further Outpatient Care

Patients with familial Mediterranean fever (FMF) should be seen regularly to ensure compliance with therapy. In one study, only 2% of 906 patients who were at high risk and compliant developed amyloidosis, compared with 49% of 54 patients who admitted noncompliance.

Teenagers are typically a noncompliant group and need long-term daily therapy to prevent chronic complications. For many of these patients, noncompliance is associated with severe symptoms, which may reinforce the need for therapy. Communicating with patients' pharmacies to determine how often they are obtaining refills may be the best way to assess compliance.

Perform a urinalysis at every visit, particularly in patients at risk of developing amyloidosis. If proteinuria is present, assess patients carefully for compliance. Exclude other causes of proteinuria (eg, heavy sports activity). In patients with hypertension, proteinuria of greater than 3.5 g/24 h and severe FMF, amyloidosis is the more likely cause. However, in nonhypertensive patients with milder diseases, other causes of proteinuria may exist and a biopsy should be considered. [15] If amyloidosis is confirmed, increase the daily dose of colchicine.

For unknown reasons, hematuria occurs in approximately 5% of patients. Its presence, along with prolonged abdominal or muscle pain, suggests the development of polyarteritis nodosa.

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Complications

Patients with amyloidosis may develop an acute onset of renal failure if they are stressed by dehydration, infection, or both.

Renal vein thrombosis may occur in nephrotic patients. This condition may manifest as abdominal or flank pain, increasing proteinuria, and worsening renal function. Acute anticoagulation may stabilize or improve renal function.

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Prognosis

Patients who are compliant with daily colchicine can probably expect to have a normal lifespan if colchicine is started before proteinuria develops.

Even with amyloidosis, the use of colchicine, dialysis, and renal transplantation should extend a patient's life beyond age 50 years.

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Patient Education

Patients with FMF need to understand the importance of strict compliance with daily colchicine therapy. Patient education information on FMF is available through the American College of Rheumatology.

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