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Familial Mediterranean fever
Definition Familial Mediterranean fever is a disorder passed down through families (inherited), which involves repeated fevers and inflammation that often affects the abdomen or lungs. Alternative Names Familial paroxysmal polyserositis; Periodic peritonitis; Recurrent polyserositis; Benign paroxysmal peritonitis; Periodic disease; Periodic fever 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
- Pain in hip, knee, ankle, shoulder elbow or wrist
- Pain over the small joints of the foot or 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. 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. They include: - White blood cell count
- Erythrocyte sedimentation rate (ESR)
- Plasma fibrinogen
- Serum haptoglobin
- Ceruloplasmin
- 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. 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. 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. Review date: 8/15/2006 Reviewed By: Hilary M. Babcock, M.D., Medical Director of Occupational Infection Control, Barnes-Jewish and St. Louis Children's Hospitals; Instructor of Medicine, Infectious Disease Division, Washington University School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network.
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