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Condition

Familial Mediterranean Fever

Condition 101

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 inflammation.

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 are the support groups for Familial Mediterranean Fever?

The following resources can provide more information on FMF:

  • American College of Rheumatology -- www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Familial-Mediterranean-Fever-Juvenile
  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/familial-mediterranean-fever
  • NLM Genetics Home Reference -- ghr.nlm.nih.gov/condition/familial-mediterranean-fever

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?

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

REFERENCES

Ombrello AK, Kastner DL. Hereditary periodic fever syndromes and other systemic autoinflammatory diseases. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 163.

Shohat M. Familial Mediterranean fever. GeneReviews. 2016; Dec 15. PMID: 20301405 www.ncbi.nlm.nih.gov/pubmed/20301405.

Data Source : ADAM

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