What is included in long-term monitoring of familial Mediterranean fever (FMF)?

Updated: May 14, 2018
  • Author: John O Meyerhoff, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

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. [17] 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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