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.
Familial paroxysmal polyserositis; Periodic peritonitis; Recurrent polyserositis; Benign paroxysmal peritonitis; Periodic disease; Periodic fever; FMF
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.
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:
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.
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.
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.
Contact your provider if you or your child develops symptoms of this condition.
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/.