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Familial 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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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:

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.
Joint attacks, occurring in large joints, mainly of the legs. Usually, only one joint is affected. 75% of all FMF patients experience joint attacks.
Chest attacks with pleuritis (inflammation of the pleural lining) and pericarditis (inflammation of the pericardium). Pleuritis occurs in 40%, but pericarditis is rare.
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)
Myalgia (rare in isolation)
Erysipeloid (a skin reaction on the legs, rare in isolation)
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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Clinical quiz
From American Family Physician, 8/1/05

Clinical Quiz questions are based on selected articles in this issue of American Family Physician. Answers appear in this issue. AFP has been reviewed by the American Academy of Family Physicians as having content acceptable for Prescribed credit. Term of approval is for one year from the beginning distribution date of January 2005. This issue has been reviewed and is acceptable for up to 3.0 Prescribed credits, of which 0.5 credit conforms to AAFP criteria for evidence-based continuing medical education (EB CME) clinical content. The amount of CME has been doubled to reflect two-for-one credit for the EB CME portion only. When reporting CME credits, AAFP members should report total Prescribed credits earned for this activity. It is not necessary for members to label credits as EB CME for reporting purposes.

The AAFP is accredited by the Accreditation Council for Continuing Medical Education to provide CME for physicians.

The AAFP designates this educational activity for a maximum of 3.0 Category 1 credits toward the American Medical Association Physician's Recognition Award (AMA/PRA). Each physician should claim only those credits that he or she actually spent in the activity.

AAFP Credit

Each copy of AFP contains a Clinical Quiz answer card. AAFP members may use this card to obtain the designated number of Prescribed credit hours for the year in which the card is postmarked.

AMA/PRA Category 1 Credit

AAFP members who satisfy the Academy's CME requirements are automatically eligible for the AMA/PRA.

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1 Read each article, answer all questions on the quiz pages, and transfer your answers to the Clinical Quiz answer card (bound into your copy of AFP). This will help you avoid errors and permit you to check your answers against the correct answers.

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Clinical Quiz questions are written by the associate and assistant editors of AFP.

CME Quality Survey

Please answer the following questions to help us monitor the quality of AFP's CME material on an ongoing basis. Mark your answers on this issue's quiz card. We would appreciate hearing any suggestions you have for improving the CME experience offered through AFP. See the directory on page 355.

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