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 may 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 having 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       o Pain in hip, knee, ankle, shoulder elbow or wrist       o Pain over the small joints of the foot or hand       o 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 may 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 may 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 may 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.

Johns Hopkins patient information

Last revised: December 3, 2012
by Martin A. Harms, M.D.

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