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Mediterranean fever

Familial Mediterranean fever (FMF) is a hereditary inflammatory disorder that affects groups of patients originating from around the Mediterranean Sea (hence its name). It is prominently present in the Armenian people (up to 1 in 7 affected), Sephardi Jews (and, to a much lesser extent, Ashkenazi Jews), people from Turkey, the Arab countries and Lebanon. more...

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Medicines

Clinical symptoms

Attacks

There are seven types of attacks. 90% of all patients have their first attack before they are 20 years old. All develop over 2-4 hours and last anytime between 6 hours and 4 days. Most attacks involve fever:

  1. Abdominal attacks, featuring abdominal pain affecting the whole abdomen with all signs of acute abdomen (e.g. appendicitis). They occur in 95% of all patients and may lead to unnecessary laparotomy. Incomplete attacks, with local tenderness and normal blood tests, have been reported.
  2. Joint attacks, occurring in large joints, mainly of the legs. Usually, only one joint is affected. 75% of all FMF patients experience joint attacks.
  3. Chest attacks with pleuritis (inflammation of the pleural lining) and pericarditis (inflammation of the pericardium). Pleuritis occurs in 40%, but pericarditis is rare.
  4. Scrotal attacks due to inflammation of the tunica vaginalis. This occurs in up to 5% and may be mistaken for acute scrotum (i.e. testicular torsion)
  5. Myalgia (rare in isolation)
  6. Erysipeloid (a skin reaction on the legs, rare in isolation)
  7. Fever without any symptoms (25%)

Complications

AA-amyloidosis with renal failure is a complication and may develop without overt crises. AA (amyloid protein) is produced in very large quantities during attacks and at a low rate between them, and accumulates mainly in the kidney, as well as the heart, spleen, gastrointestinal tract and the thyroid.

There appears to be an increase in the risk for developing particular vasculitis-related diseases (e.g. Henoch-Schoenlein purpura), spondylarthropathy, prolonged arthritis of certain joints and protracted myalgia.

Diagnosis

The diagnosis is clinically made on the basis of the history of typical attacks, especially in patients from the ethnic groups in which FMF is more highly prevalent. An acute phase response is present during attacks, with high C-reactive protein levels, an elevated white blood cell count and other markers of inflammation. In patients with a long history of attacks, monitoring the renal function is of importance in predicting chronic renal failure.

A genetic test is also available now that the disease has been linked to mutations in the MEFV gene. Sequencing of exons 2, 3, 5, and 10 of this gene detects an estimated 97% of all known mutations.

Disease mechanism

Pathophysiology

Virtually all cases are due to a mutation in the MEFV gene, which codes for a protein called pyrin or marenostenin. This was discovered in 1997 by two different groups. Various mutations of this gene lead to FMF, although some mutations cause a more severe picture than others. Mutations occur in exons 2, 3, 5 and 10.

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Relapsing fever
From Gale Encyclopedia of Medicine, 4/6/01 by Rosalyn S. Carson-DeWitt

Definition

Relapsing fever refers to two similar illnesses, both of which cause high fevers. The fevers resolve, only to recur again within about a week.

Description

Relapsing fever is caused by spiral-shaped bacteria of the genus Borrelia. This bacterium lives in rodents and in insects, specifically ticks and body lice. The form of relapsing fever acquired from ticks is slightly different from that acquired from body lice.

In tick-borne relapsing fever (TBRF), rodents (rats, mice, chipmunks, and squirrels) which carry Borrelia are fed upon by ticks. The ticks then acquire the bacteria, and are able to pass it on to humans. TBRF is most common in sub-Saharan Africa, parts of the Mediterranean, areas in the Middle East, India, China, and the south of Russia. Also, Borrelia causing TBRF exist in the western regions of the United States, particularly in mountainous areas. The disease is said to be endemic to these areas, meaning that the causative agents occur naturally and consistently within these locations.

In louse-borne relapsing fever (LBRF), lice acquire Borrelia from humans who are already infected. These lice can then go on to infect other humans. LBRF is said to be epidemic, as opposed to endemic, meaning that it can occur suddenly in large numbers in specific communities of people. LBRF occurs in places where poverty and overcrowding predispose to human infestation with lice. LBRF has flared during wars, when conditions are crowded and good hygiene is next to impossible. At this time, LBRF is found in areas of east and central Africa, China, and in the Andes Mountains of Peru.

Causes & symptoms

In TBRF, humans contract Borrelia when they are fed upon by ticks. Ticks often feed on humans at night, so many people who have been bitten are unaware that they have been. The bacteria is passed on to humans through the infected body fluids of the tick.

In LBRF, a louse must be crushed or smashed in order for Borrelia to be released. The bacteria then enter the human body through areas where the person may have scratched him or herself.

Both types of relapsing fever occur some days after having acquired the bacteria. About a week after becoming infected, symptoms begin. The patient spikes a very high fever, with chills, sweating, terrible headache, nausea, vomiting, severe pain in the muscles and joints, and extreme weakness. The patient may become dizzy and confused. The eyes may be bloodshot and very sensitive to light. A cough may develop. The heart rate is greatly increased, and the liver and spleen may be swollen. Because the substances responsible for blood clotting may be disturbed during the illness, tiny purple marks may appear on the skin, which are evidence of minor bleeding occurring under the skin. The patient may suffer from a nosebleed, or may cough up bloody sputum. All of these symptoms last for about three days in TBRF, and about five days in LBRF.

With or without treatment, a crisis may occur as the bacteria are cleared from the blood. This crisis, called a Jarisch-Herxheimer reaction, results in a new spike in fever, chills, and an initial rise in blood pressure. The blood pressure then falls drastically, which may deprive tissues and organs of appropriate blood flow (shock). This reaction usually lasts for about a day.

Recurrent episodes of fever with less severe symptoms occur after about a week. In untreated infections, fevers recur about three times in TBRF, and only once or twice in LBRF.

Diagnosis

Diagnosis of relapsing fever is relatively easy, because the causative bacteria can be found by examining a sample of blood under the microscope. The characteristically spiral-shaped bacteria are easily identifiable. The blood is best drawn during the period of high fever, because the bacteria are present in the blood in great numbers at that time.

Treatment

Either tetracycline or erythromycin is effective against both forms of relapsing fever. The medications are given for about a week for cases of TBRF; LBRF requires only a single dose. Children and pregnant women should receive either erythromycin or penicillin. Because of the risk of the Jarish-Herxheimer reaction, patients must be very carefully monitored during the initial administration of antibiotic medications. Solutions containing salts must be given through a needle in the vein (intravenously) to keep the blood pressure from dropping too drastically. Patients with extreme reactions may need medications to improve blood circulation until the reaction resolves.

Prognosis

In epidemics of LBRF, death rates among untreated victims have run as high as 30%. With treatment, and careful monitoring for the development of the Jarish-Herxheimer reaction, prognosis is good for both LBRF and TBRF.

Prevention

Prevention of TBRF requires rodent control, especially in and near homes. Careful use of insecticides on skin and clothing is important for people who may be enjoying outdoor recreation in areas known to harbor the disease-carrying ticks.

Prevention of LBRF is possible, but probably more difficult. Good hygiene and decent living conditions would prevent the spread of LBRF, but these may be difficult for those people most at risk for the disease.

Key Terms

Endemic
Refers to a particular organism which consistently exists in a particular location under normal conditions.
Epidemic
Refers to a condition suddenly acquired by a large number of people within a specific community, and which spreads rapidly throughout that community.
Shock
A state in which the blood pressure is so low that organs and tissues are not receiving an appropriate flow of blood.

Further Reading

For Your Information

    Books

  • Dennis, David T., and Grant L. Campbell. "Relapsing Fever." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
  • Sherris, John C., and James J. Plorde. "Spirochetes." In Sherris Medical Microbiology: An Introduction to Infectious Diseases, edited by Kenneth J. Ryan. Norwalk, CT: Appleton and Lange, 1994.

    Periodicals

  • Anda, Pedro, et al. "A New Borrelia Species Isolated From Patients with Relapsing Fever in Spain." The Lancet, 348, no. 9021 (July 20, 1996): 162+.
  • Cooper, Randy I., and Thomas Neuhauser. "Borreliosis." The New England Journal of Medicine, 338, no. 4 (January 22, 1998): 231.
  • Newton, James A., and Patricia V. Pepper. "Relapsing Fever." The New England Journal of Medicine 335, no. 16 (October 17, 1996): 1197.

    Organizations

  • Centers for Disease Control and Prevention. 1600 Clifton Road NE, Atlanta, GA 30333. (404) 332-4559. http://www.cdc.gov.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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