Main Campus: Chesterfield, MO 63017 | 
Familial Mediterranean fever
Familial paroxysmal polyserositis; Periodic peritonitis; Recurrent polyserositis; Benign paroxysmal peritonitis; Periodic disease; Periodic fever; FMF
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.
Causes
FMF is most often caused by a mutation in a gene named MEFV . This gene creates proteins involved in inflammation.
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
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:
-
Abdominal pain
Abdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
ImageRead Article Now Book Mark Article - Chest pain that is sharp and gets worse when taking a breath
- Fever or alternating chills and fever
- Joint pain
- Skin sores (lesions) that are red and swollen and range from 5 to 20 cm in diameter
Exams and Tests
If genetic testing shows that you have the MEFV gene mutation and your symptoms match the typical pattern, the diagnosis is nearly certain. Laboratory tests or x-rays can rule out other possible diseases to help make the diagnosis.
x-rays
X-rays are a type of electromagnetic radiation, just like visible light. An x-ray machine sends individual x-ray particles through the body. The im...
Levels of certain blood tests may be higher than normal when done during an attack. Tests may include:
-
Complete blood count
(CBC)
Complete blood count
A complete blood count (CBC) test measures the following:The number of red blood cells (RBC count)The number of white blood cells (WBC count)The tota...
ImageRead Article Now Book Mark Article -
C-reactive protein
to check for inflammation
C-reactive protein
C-reactive protein (CRP) is produced by the liver. The level of CRP rises when there is inflammation throughout the body. It is one of a group of p...
ImageRead Article Now Book Mark Article -
Erythrocyte sedimentation rate
(ESR) to check for inflammation
Erythrocyte sedimentation rate
ESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures how much inflammation is in...
Read Article Now Book Mark Article -
Fibrinogen test
to check blood clotting
Fibrinogen test
Fibrinogen is a protein produced by the liver. This protein helps stop bleeding by helping blood clots to form. A blood test can be done to tell ho...
Read Article Now Book Mark Article -
White blood cell count
White blood cell count
A WBC count is a blood test to measure the number of white blood cells (WBCs) in the blood. WBCs help fight infections. They are also called leukocy...
ImageRead Article Now Book Mark Article
Treatment
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.
Amyloidosis
Primary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid depo...
NSAIDs may be used to treat fever and pain.
Outlook (Prognosis)
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.
Possible Complications
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.
When to Contact a Medical Professional
Call your provider if you or your child develops symptoms of this condition.
References
Ombrello AK, Kastner DL. Hereditary periodic fever syndromes and other systemic autoinflammatory diseases. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 163.
Simon A, van der Meer JWM, Drenth JPH. Familial autoinflammatory syndromes. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelly's Textbook of Rheumatology . 9th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 97.
-
Temperature measurement - illustration
A thermometer is a useful aid used to measure body temperature. A thermometer is usually filled with mercury. Mercury in the tube rises when expanded by an increase in body temperature.
Temperature measurement
illustration
-
Fever of unknown origin
(Alt. Medicine)
Review Date: 7/31/2016
Reviewed By: Jatin M. Vyas, MD, PhD, Assistant Professor in Medicine, Harvard Medical School; Assistant in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
