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Familial Mediterranean fever


Alternative names

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

Definition

Familial Mediterranean fever is an inherited disorder characterized by recurrent fever and inflammation, often involving the abdomen or the lung.

Causes, incidence, and risk factors

The cause of familial Mediterranean fever is unknown. It usually affects people of Mediterranean ancestry, especially non-Ashkenazi (Sephardic) Jews, Armenians, and Arabs, although people from other ethnic groups can also be affected.

Symptoms usually begin between age 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, and people are usually symptom free between attacks.

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

Symptoms

  • Fever or alternating chills and fever (relapsing)
  • Abdominal pain
  • Chest pain that occurs repeatedly (recurrent)
  • Recurrent abdominal pain, recurrent
  • Recurrent joint pain
    • Hip pain ,
    • Knee pain
    • Ankle pain
    • Foot pain , pain over the small joints of the foot
    • Shoulder pain
    • Elbow pain
    • Wrist pain
    • Hand pain, pain over the small joints of the hand
    • Pain in other joints
  • Skin lesions that are red and swollen and range from 5-20 cm in diameter

Signs and tests

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

Patients with familial Mediterranean fever may have any of the following during an attack:

  • Elevated white blood cell count
  • Elevated erythrocyte sedimentation rate ( ESR )
  • Elevated plasma fibrinogen
  • Elevated serum haptoglobin
  • Elevated ceruloplasmin
  • Elevated C-reactive protein

Treatment

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

Expectations (prognosis)

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.

Complications

  • Discomfort is the primary complication.
  • Narcotic addiction can sometimes occur, but addiction rates are not higher than for the general population if the pain associated with the condition is recognized and treated appropriately.
  • Gallbladder disease can also occur.
  • Amyloidosis (deposits of protein in different organs) is more common in patients with familial Mediterranean fever.

Calling your health care provider

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.

Update Date: 7/31/2002

Christopher Parsons, M.D., Department of Internal Medicine, Division of Infectious Diseases, University of Virginia, Charlottesville, VA. Review provided by VeriMed Healthcare Network.

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Last updated: Tue, 06 Jan 2009 00:20:03 GMT
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