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Familial paroxysmal polyserositis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Familial Mediterranean fever (FMF) is characterized be episodes of fever, peritonitis and/or pleuritis

  • is an autosomal-recessive, hereditary auto-inflammatory disease and has a reference in the Online Mendelian Inheritance in Man database (OMIM) ID: 249100
  • FMF occurs as a result of mutations in the MEditerranean FeVer (MEFV gene)
    • is the only gene currently known to be associated with FMF and is located on chromosome 16
  • primary characteristic of FMF is
    • recurrent fever and serositis
      • which results in pain in the abdomen, chest, joints, muscles, etc.
    • amyloidosis, skin lesions and arthritis may be seen in these patients
      • the most severe complication of FMF is amyloid A (AA) amyloidosis leading to renal failure

This condition occurs most often in non-Ashkenazi Jews, Armenians and Arabs. It is not restricted to these groups and may occur in, among others, people of Ashkenazi Jewish, Italian, and Anglo-saxon descent.

There are mainly two phenotypes in FMF

  • Type 1 is commonly associated with recurrent short episodes of inflammation and serositis, including fever, peritonitis, synovitis, pleuritis, and rarely
    pericarditis and meningitis
    • symptoms and severity vary from one person to another
    • typical clinical manifestations of FMF type 1 usually last from 12 to 72 hours and include the following typical attacks (2):
      • 1) recurrent fever, characterized by a temperature ranging from 38 degrees C to 40 C;
      • 2) abdominal attacks, featuring abdominal pain (usually the entire abdomen is involved);
      • 3. arthritic attacks, frequently featuring as monoarthritis localized in the large joints of the leg (hip, knee, ankle);
      • 4. chest attacks, including pleuritis and pericarditis;
      • 5. pre-attack symptoms, occurring 12 to 24 hours before any FMF attacks, usually including discomfort, abnormal taste sensation,
        dizziness, increased appetite, irritability, etc.
  • Type 2 FMF is characterized by amyloidosis as the first clinical manifestation of the disease, in otherwise asymptomatic people

A systematic review with respect to interventions in familial Mediterranean fever (1):

  • based on limited evidence, review (10 RCTs; n= 312) found three times daily colchicine may reduce number of attacks and outcomes after single dose and divided doses may not be different in children
  • for colchicine-resistant disease, anakinra and canakinumab might be effective

Reference:

  • Yin X, Tian F, Wu B, Xu T. Interventions for reducing inflammation in familial Mediterranean fever. Cochrane Database of Systematic Reviews 2022, Issue 3. Art. No.: CD010893. DOI: 10.1002/14651858.CD010893.pub4
  • Lidar M et al.The prodrome: aprominent yet overlooked preattack manifestation of familial Mediterranean fever. Journal of Rheumatology 2006;33(6):1089-92.

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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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