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What is Familial Mediterranean Fever?

Monday 29 June 2020
Gout

Table of Contents


I. What is Familial Mediterranean Fever?

II. Symptoms of FMF

a. Complications

III. What Causes FMF?

IV. How is FMF Treated?


What is Familial Mediterranean Fever?

Familial Mediterranean fever (FMF) is an inflammatory disorder that can cause severe pain in the joints, lungs, and abdomen. FMF is an inherited condition that typically affects people of Mediterranean descent, including people of Greek, Italian, Jewish, Arab, Turkish, Armenian, or North African ancestry. In these populations, FMF affects around 1 in every 200 people. [1] [2]

FMF causes recurring episodes of inflammation. Around half of FMF patients show mild signs before a painful episode. Episodes can vary in their length and severity, with most episodes lasting between 12 and 72 hours. [3] Episodes can also differ in their frequency. Some patients may suffer regular episodes, while others may go for years without a flare-up. Usually, FMF patients show no other signs or symptoms in between episodes.

Keep reading to learn about what causes FMF and how it can be treated with medications like Colcrys (colchicine).

Symptoms of FMF

Familial Mediterranean fever causes episodes or attacks that can cause severe pain. FMF causes inflammation of the membranes that surround joints, lungs, and abdomen, although it can affect other areas of the body.

As well as severe pain, FMF may also cause other symptoms, including:

  • Fever
  • Painful rashes on the lower parts of the legs or feet
  • Swollen scrotum
  • Muscle aches
  • Spleen enlargement
  • Mild abdominal bloating
  • Tight or rigid abdominal muscles
  • Swelling in the fingers and toes [4]

A depiction of skin rashes on the wrist

Some patients experience mild signs before an episode. These are called prodrome symptoms. Prodromal symptoms include general disagreeable feelings and uncomfortable sensations in the areas that will become inflamed.

Most of these symptoms subside within a week of the episode, although occasionally some pain or join function can continue for several weeks following an FMF attack.

Often, the initial symptom of FMF is a recurrent fever during childhood. These fevers cause temperatures to spike to over 100 degrees Fahrenheit. [2]

a. Complications

When FMF is not treated, it can cause serious complications. In women, FMF inflammation can affect reproductive organs and cause infertility. Complications can also cause kidney damage, increased joint pain or an abnormal amount of protein in the blood, which can cause organ damage. [6]

What Causes Familial Mediterranean Fever?

Familial Mediterranean fever typically begins during childhood or adolescence. Approximately three-quarters of FMF patients have their first episode before they turn 10 and 90 percent of patients before they turn 20. [5]

 It is an inherited disorder that is the result of a gene mutation. It is very likely that patients with FMF will have someone else in their extended family that also has the condition. [5]

It is the mutation in the gene MEFV that affects the severity of the condition. [6] MEFV creates the protein pyrin in the body. Pyrin then controls inflammation. Usually, inflammation occurs when tissue in the body is injured or damaged. Skin cells release chemicals that result in fluid leaking into skin tissue to create swelling. [7] When the MEFV gene is mutated, pyrin’s activity is reduced. This results in extended or unnecessary inflammation in the body.

Some patients that only inherit one mutated gene may also develop symptoms of other inflammatory diseases, including Crohn’s disease or Behcet’s disease.

It is not known what triggers an episode of familial Mediterranean fever. However, it is thought that triggers can include menstruation, stress, colds, infections, and medications. Some episodes may occur spontaneously without an obvious trigger. [2]

A woman holds her head in despair

How is FMF Treated?

Familial Mediterranean fever is a chronic disorder with no cure. However, medications can help to relieve the pain and symptoms of this condition. The first step to diagnosing FMF is a review of your family’s medical history. This is important as this condition is often passed from parents to children. After a review of your medical history, doctors may take blood tests to check for an increase in white blood cells or complete genetic testing to check for the MEFV gene. However, it is impossible to check for every gene mutation associated with FMF so this may return a false-negative result. [6]

A tablet container with many different medication pills

A common medication for patients with FMF is Colcrys (colchicine). Colcrys is prescribed for a number of conditions that involve pain caused by inflammation, including gout and Behcet’s disease. When treating FMF, Colcrys works by decreasing the amount of the protein amyloid A that the body produces. [8] This helps to reduce swelling and pain caused by inflammation. Studies suggest that over 90 percent of patients that take colchicine for FMF show an improvement in the length and frequency of their episodes. [2] People with FMF may have to take colchicine for the rest of their life. Speak to a doctor about the best method and frequency of dosing.

As well as colchicine, FMF patients may also benefit from taking nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin, ibuprofen, or naproxen to relieve joint and muscle pain.

The content in this article is intended for informational purposes only. This website does not provide medical advice. In all circumstances, you should always seek the advice of your physician and/or other qualified health professionals(s) for drug, medical condition, or treatment advice. The content provided on this website is not a substitute for professional medical advice, diagnosis or treatment.