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Familial Mediterranean fever

Contents of this page:

Illustrations

Temperature measurement
Temperature measurement

Alternative Names    Return to top

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

Definition    Return to top

Familial Mediterranean fever is a disorder passed down through families (inherited), which involves repeated fevers and inflammation that often affects the abdomen or lungs.

Causes    Return to top

Familial Mediterranean fever is caused by a mutation in the MEFV gene. The condition usually affects people of Mediterranean ancestry, especially non-Ashkenazi (Sephardic) Jews, Armenians, and Arabs, although people from other ethnic groups may also be affected.

This disease is very rare. Risk factors include a family history of familial Mediterranean fever or having Mediterranean ancestry.

Symptoms    Return to top

Symptoms usually begin 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. Patients are usually symptom-free between attacks.

Symptoms may include:

Exams and Tests    Return to top

There is no specific test to diagnose this disease. Sometimes analysis of the chromosomes can help. Ruling out other possible diseases using laboratory tests or x-rays will help determine the diagnosis.

Certain blood tests may be higher-than-normal when done during an attack. Tests may include:

Treatment    Return to top

The treatment for familial Mediterranean fever is treatment of symptoms. Colchicine, a medicine that reduces inflammation, may help during an attack and may prevent further attacks.

Outlook (Prognosis)    Return to top

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

Possible Complications    Return to top

Discomfort is the primary complication. There is a risk of addiction to the narcotic pain relievers used to relieve symptoms.

Amyloidosis (deposits of protein in different organs) is more common in patients with familial Mediterranean fever. Gallbladder disease may also occur.

When to Contact a Medical Professional    Return to top

Call your health care provider if symptoms develop to rule out other possible causes and get appropriate treatment. See a pain specialist if there is chronic pain.

References    Return to top

Kastner DL. The systemic autoinflammatory diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 282.

Update Date: 9/28/2008

Updated by: Jatin M. Vyas, PHD, MD, Instructor in Medicine, Harvard Medical School, Assistant in Medicine, Division of Infectious Disease, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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