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Familial Mediterranean fever

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of familial Mediterranean fever are prepared by our editorial team based on guidelines from the Egyptian Expert Group on Familial Mediterranean Fever (FMF-EEG 2022), the Brazilian Society of Rheumatology (BSR 2016), the European League Against Rheumatism (EULAR 2016), the American Academy of Allergy, Asthma & Immunology (AAAAI/ACAAI 2015), and the Single ...
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Screening and diagnosis

Diagnosis: as per AAAAI/ACAAI 2015 guidelines, suspect FMF in patients presenting with recurrent and often prolonged fever attacks associated with serosal, cutaneous, and synovial manifestations.
B
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  • Diagnosis (RBR)

Diagnostic investigations

Clinical assessment: as per AAAAI/ACAAI 2015 guidelines, assess for persistent systemic signs of inflammation in patients with suspected FMF in the absence of demonstrable infection or autoimmune disease.
B

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  • Laboratory tests

  • Genetic testing

  • Genetic testing (RBR)

Medical management

General principles, EEG-FMF: as per FMF-EEG 2022 guidelines, consider identifying possible triggers to help in preventing possible attacks by temporarily increasing the colchicine dose.
C

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  • General principles

  • Colchicine

  • Colchicine (RBR)

  • Colchicine (EEG-FMF)

  • NSAIDs

  • NSAIDs (EEG-FMF)

  • Management of protracted febrile myalgia

  • Management of protracted febrile myalgia (EEG-FMF)

  • Management of resistant disease

  • Management of resistant disease (RBR)

  • Management of resistant disease (EEG-FMF)

  • Management of chronic arthritis

  • Management of chronic arthritis (EEG-FMF)

  • Management of AA amyloidosis

Specific circumstances

Pregnant patients
As per EULAR 2016 guidelines:
Do not discontinue colchicine during conception, pregnancy, or lactation. Do not perform amniocentesis.
D
Do not discontinue colchicine before conception in male patients. Consider reducing the dose or discontinuing temporarily in the rare case of azoospermia or oligospermia proven to be related to colchicine.
D

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  • Pregnant patients (EEG-FMF)

  • Pediatric patients

Follow-up and surveillance

Assessment of treatment response
As per FMF-EEG 2022 guidelines:
Monitor patients for 3-6 months to assess for the therapeutic effect on attack frequency and severity (clinical diary and acute phase reactants).
B
Consider extending the follow-up frequency up to 1-year in controlled compliant patients.
C

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  • Assessment of treatment compliance

  • Dose adjustments (EEG-FMF)

  • Dose adjustments

  • Monitoring for treatment-related adverse effects

  • Monitoring for treatment-related adverse effects (EEG-FMF)