Treatment of colchicine resistant Familial Mediterranean fever (FMF) with Anakinra

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Treatment of colchicine resistant Familial
Mediterranean fever (FMF) with Anakinra

• Javadi parvaneh
  Vadood
• M.D. , Fellow of pediatric rheumatology, Mofid
  Children's Hospital
• Shiari
  Reza
• M. D., PhD. , Associate Professor of Pediatric
  Rheumatology, Mofid Children's Hospital, Shahid
  Beheshti University of Medical Sciences

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FMF
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Autosomal recessive
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The most common hereditary periodic fever
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Diagnosis is still based on clinical manifestation
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Genetic testing used as a confirmatory test
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• Detailed criteria for the diagnosis of familial
  Mediterranean fever
• Major criteriaTypical attacks
• 1. Peritonitis (generalized)
• 2. Pleuritis (unilateral) or pericarditis
• 3. Monoarthritis (hip, knee, ankle)
• 4. Fever alone
• 5. Favorable response to colchicine
• Minor criteria
• 1-4. Incomplete attacks involving one or more of
  the following sites
• 1. Abdomen 2. Chest 3. Joint 4. Exertional leg
  pain
• Supportive criteria
• 1. Family history of FMF
• 2. Appropriate ethnic origin
• 3. Age lt20 years at disease onset
• 4-7. Features of attacks
• 4. Severe, requiring bed rest 5. Spontaneous
  remission 6 . Symptom-free interval
• 7. Transient inflammatory response, with one or
  more abnormal test result(s) for the white blood
  cell count, erythrocyte sedimentation rate, serum
  amyloid A, and/or fibrinogen
• 8. Episodic proteinuria/hematuria

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FMF
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FMF
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• COLCHICINE
• 90-95
• RESPONDER

5-10 Nonresponder or intolerant
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FMF
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Prazosin
Etanercept
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FMF
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Targets type1IL-1 receptor which is expressed in
many tissues
IL-1 receptor antagonist
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FMF
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FMF
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M694V,R761H
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Rx
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from 3 yrs ago
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FMF
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• Efficacy of anakinra treatment in a patient with
  colchicine-resistant familial Mediterranean fever
• Alpay,Abdullah Sumnu,Yasar CaliskanNilufer

For 2 months F/U
No nausea and vomiting

• Anakinra suppresses familial Mediterreanean fever
  in a colchicine-resistant patient
• I. Mitroulis,V.P. Papadopoulos,T.Constantinidis,K.
  Tinidis

3 mild attack(duration5-10 min)
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Conclusion
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• Addition of anakinra to colchicine could be
  effective to decrease the number and severity of
  abdominal pain attacks in Colchicine resistant
  patients with FMF

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Thank You !
THANK FOR YOUR ATTENTION