What is the definition of Familial Mediterranean Fever?

Familial Mediterranean fever (FMF) is a rare disorder passed down through families (inherited). It involves repeated fevers and inflammation that often affects the lining of the abdomen, chest, or joints.

What are the alternative names for Familial Mediterranean Fever?

Familial paroxysmal polyserositis; Periodic peritonitis; Recurrent polyserositis; Benign paroxysmal peritonitis; Periodic disease; Periodic fever; FMF

What are the causes for Familial Mediterranean Fever?

FMF is most often caused by a mutation in a gene named MEFV. This gene creates a protein involved in controlling inflammation. The disease appears only in people who received two copies of the altered gene, one from each parent. This is called autosomal recessive.

FMF most often affects people of Mediterranean ancestry. These include non-Ashkenazi (Sephardic) Jews, Armenians, and Arabs. People from other ethnic groups can also be affected.

What are the symptoms for Familial Mediterranean Fever?

Symptoms usually begin between ages 5 and 15. Inflammation in the lining of the abdominal cavity, chest cavity, skin, or joints occurs along with high fevers that usually peak in 12 to 24 hours. Attacks may vary in severity of symptoms. People are usually symptom-free between attacks.

Symptoms may include repeated episodes of:

  • Abdominal pain
  • Chest pain that is sharp and gets worse when taking a breath
  • Fever or alternating chills and fever
  • Joint pain
  • Skin sores (lesions) that are red and swollen and range from 5 to 20 cm in diameter

What are the current treatments for Familial Mediterranean Fever?

The goal of treatment for FMF is to control symptoms. Colchicine, a medicine that reduces inflammation, may help during an attack and may prevent further attacks. It can also help prevent a serious complication called systemic amyloidosis, which is common in people with FMF.

NSAIDs may be used to treat fever and pain.

What is the outlook (prognosis) for Familial Mediterranean Fever?

There is no known cure for FMF. Most people continue to have attacks, but the number and severity of attacks is different from person to person.

What are the possible complications for Familial Mediterranean Fever?

Amyloidosis may lead to kidney damage or not being able to absorb nutrients from food (malabsorption). Fertility problems in women and men and arthritis are also complications.

When should I contact a medical professional for Familial Mediterranean Fever?

Contact your provider if you or your child develops symptoms of this condition.

Temperature

REFERENCES

Verbsky JW. Hereditary periodic fever syndromes and other systemic autoinflammatory diseases. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 188.

Shohat M. Familial Mediterranean fever. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, eds. GeneReviews [Internet]. University of Washington, Seattle, WA: 2000 Aug 8 [updated 2016 Dec 15]. PMID: 20301405 www.pubmed.ncbi.nlm.nih.gov/20301405/.

Clinical Trial
  • Status: Enrolling by invitation
  • Phase: N/A
  • Intervention Type: Other
  • Participants: 30
  • Start Date: December 20, 2020
Comparison the Effectiveness of Online Aerobic Dance Exercises Versus Physical Activity Counseling in Patients With Juvenile Familial Mediterranean Fever
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  • Status: Not yet recruiting
  • Intervention Type: Biological
  • Participants: 160
  • Start Date: November 2020
Characterization of a Functional Test for Mediterranean Family Fever Screening - 2