Familial Mediterranean fever (FMF)FFamilial Mediterranean fever (FMF)Familial Mediterranean fever (FMF)EnglishRheumatologyToddler (13-24 months);Preschooler (2-4 years);School age child (5-8 years);Pre-teen (9-12 years);Teen (13-18 years)Chest;Abdomen;Knee;Ankle;SkinImmune systemConditions and diseasesCaregivers Adult (19+)NA2015-07-15T04:00:00ZRon Laxer, MD, FRCPC;Karyl S. Barron, MD (National Institutes of Health)​​10.000000000000053.0000000000000950.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Familial Mediterranean fever (FMF) involves recurrent episodes of fever accompanied by symptoms of abdominal pain, chest pain, joint pain and/or skin rashes. Learn about causes, tests and treatments for FMF.</p><p>Familial Mediterranean fever (FMF) is a disease that involves recurrent episodes of <a href="/Article?contentid=30&language=English">fever</a> without an associated infection. Attacks of fever in FMF are usually accompanied by symptoms of inflammation in one or more sites. These may include abdominal pain, chest pain, joint pain and skin rashes, among others. It is a life-long disease and there is no known cure. Fortunately, however, it can be controlled with treatment.</p><h2>Key points</h2> <ul> <li>FMF is a disease that causes regular attacks of fever.</li> <li>The attacks are accompanied by inflammation in areas such as the abdomen, chest, joints and skin.</li> <li>FMF can be treated and controlled with a medication called colchicine.</li> </ul><h2>Symptoms of FMF</h2> <p>A typical attack of FMF starts with a sudden rise in temperature, often up to 104°F (40°C). Fever usually lasts from one to three days and in most cases is accompanied by severe abdominal pain. The pain comes from inflammation of the lining of the abdomen. Sometimes the pain is so bad that it seems as if the child has <a href="/Article?contentid=818&language=English">appendicitis</a>, even though the appendix itself is not inflamed. Severe chest pain, called pleuritis, occurs in about one-third of patients. Brief episodes of arthritis, usually involving the knee or ankle, can occur in about half of patients. About one-third of patients get painful red skin and swelling, called erysipelas. This typically occurs over the foot. These symptoms generally disappear as the fever resolves, although attacks of arthritis may last for up to one week. Rarely, arthritis affecting a single joint, including the joints of the back (sacroiliitis), may persist. Attacks may also be accompanied by severe muscle pain, called myalgia.</p><h2>How common is FMF?</h2> <p>FMF is more common in certain ethnic groups, primarily those whose ancestry was around the Mediterranean Sea. These groups include Armenians, Turks, Arabs and Jews, especially of Sephardic background. More recently, however, FMF has been diagnosed in people from many other ethnic backgrounds. Attacks of FMF begin before the age of 20 in 90% of patients. Attacks begin before age 10 in at least 50% of patients.</p> <h2>Causes of FMF</h2> <p>FMF appears to be caused by failure of the body to control the inflammation system. A protein called pyrin is an important controller of inflammation in the body. Changes (mutations) in the gene that makes pyrin, called MEFV, are found in up to 80% of FMF cases. Most cases require two mutations (one from the mother and one from the father) in the MEFV gene to result in FMF. This type of disease is called “autosomal recessive,” meaning that parents are generally carriers. There is often, but not always, a history of another family member having a similar disease. Sometimes only one or even no mutations are found in typical cases of FMF.</p><h2>Treatment of FMF</h2> <p>Fortunately, amyloidosis can almost always be prevented by stopping attacks of inflammation with the drug colchicine. Taking colchicine every day reduces the frequency of the attacks as well as the severity of the attacks that do occur, and therefore prevents the development of amyloidosis. Colchicine is prescribed as a daily medication. In some patients, missing even one dose may result in an attack. You and your doctor will decide if your child needs colchicine and if so, for how long. </p> <h2>Lab tests for children with FMF</h2> <p>During the attacks, blood tests will usually show very marked degrees of inflammation. The most commonly requested tests are a complete blood count (CBC), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). These levels return to normal or near normal between attacks.</p> <p>Your doctor will check for the development of amyloidosis by performing a general physical examination and also a urine test to check for protein in the urine every six months.</p><h2>Complications of FMF</h2> <p>Without treatment, attacks of FMF will occur frequently and can be disabling for the patient. If attacks of inflammation are not controlled, there is a risk of developing amyloidosis, the most serious complication of FMF. In amyloidosis, a protein of inflammation called SAA is deposited in multiple organ systems, particularly the kidney, heart and gastrointestinal tract. Amyloidosis appears to be less common in patients born and raised in the northern hemisphere.</p>
Fièvre méditerranéenne familiale (FMF)FFièvre méditerranéenne familiale (FMF)Familial Mediterranean fever (FMF)FrenchRheumatologyToddler (13-24 months);Preschooler (2-4 years);School age child (5-8 years);Pre-teen (9-12 years);Teen (13-18 years)Chest;Abdomen;Knee;Ankle;SkinImmune systemConditions and diseasesCaregivers Adult (19+)NA2015-07-15T04:00:00ZRon Laxer, MD, FRCPC;Karyl S. Barron, MD (National Institutes of Health)​​10.000000000000053.0000000000000950.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Renseignez-vous sur les causes de la fièvre méditerranéenne familiale (FMF), les examens qu’elle exige et ses traitements.</p><p>La fièvre méditerranéenne familiale (FMF) est une maladie enclenchant des poussées de fièvre périodiques qui n'est pas causée par une infection. Chez les personnes atteintes, les poussées de fièvre sont habituellement accompagnées de symptômes d’inflammation dans au moins une partie de l’organisme dont, entre autres, des douleurs à l’abdomen, à la poitrine et aux articulations et des éruptions cutanées. La FMF est une maladie qui dure toute la vie. Il n’existe aucun moyen connu de la guérir. Son traitement permet heureusement de la maîtriser.</p><h2>À retenir</h2> <ul> <li>La FMF est une maladie qui déclenche des poussées de fièvre périodiques.</li> <li>Les poussées de fièvre sont accompagnées d’inflammation dans les parties du corps comme l’abdomen, la poitrine, les articulations et la peau.</li> <li>La FMF peut être traitée et maîtrisée à l’aide d’un médicament appelé colchicine.</li> </ul><h2>Symptômes de la FMF</h2> <p>Une crise habituelle de FMF se manifeste d’abord par une poussée soudaine de fièvre, laquelle atteint souvent 104 °F (40 °C). La fièvre dure habituellement d’un à trois jours, et elle est le plus souvent accompagnée de fortes douleurs abdominales. Ces douleurs sont causées par l’inflammation de la paroi interne de l’abdomen. Elles sont parfois si intenses que l’enfant semble atteint d’une appendicite même si l’appendice même n’est pas enflammé. Environ le tiers des enfants présentent une pleurite (inflammation de la paroi interne du thorax) qui entraîne des douleurs intenses à la poitrine. Dans environ la moitié des cas, on observe de brèves crises d’arthrite qui touchent généralement le genou ou la cheville. Environ le tiers des enfants présentent un érysipèle (ou érésipèle) qui est un trouble cutané douloureux provoquant le rougissement et l’enflure de la peau et touche habituellement le pied. D’ordinaire, ces symptômes disparaissent lorsque la fièvre prend fin, bien que les crises d’arthrite puissent durer jusqu’à une semaine. L’arthrite qui se limite à une seule articulation, dont celle du dos (articulation sacro-iliaque) peut persister, bien que cela soit rare. Les crises peuvent également être accompagnées d’importantes douleurs musculaires appelées myalgie.</p><h2>Dans quelle mesure la FMF est-elle courante?</h2> <p>La FMF est plus fréquente au sein de certains groupes ethniques originaires des pays méditerranéens. Il s’agit des Arméniens, des Turcs, des Arabes et des Juifs, en particulier des juifs de familles sépharades. Toutefois, plus récemment, la FMF a été diagnostiquée chez de nombreuses autres origines ethniques. Les crises de FMF commencent avant l’âge de 20 ans dans 90 % des cas et avant l’âge de 10 ans dans au moins 50 % d’entre eux.</p> <h2>Causes de la FMF</h2> <p>La FMF semble être déclenchée parce que l’organisme n’arrive pas à maîtriser le système inflammatoire. Une protéine du nom de pyrine est un important régulateur de l’inflammation dans l’organisme. Des mutations (changements) du gène responsable de la production de pyrine, appelé MEFV, sont constatées dans un pourcentage élevé (jusqu’à 80 %) des cas. En général, il faut deux mutations du gène MEFV, l'une provenant du père et l'autre de la mère, pour être atteint de FMF. Il s’agit d’une maladie à transmission « autosomique récessive », ce qui signifie que les parents sont généralement porteurs. Un autre membre de la famille est souvent (mais non pas nécessairement) atteint d’une maladie similaire. Il arrive aussi que des cas caractéristiques de FMF ne soient associés qu’à une seule voire aucune mutation.</p><h2>Traitement de la FMF</h2> <p>Il est heureusement presque toujours possible de prévenir l’amyloïdose en faisant cesser les crises d’inflammation à l’aide du médicament appelé colchicine. La prise de colchicine chaque jour réduit la fréquence des crises ainsi que leur intensité, ce qui, par conséquent, permet d'éviter l’amyloïdose. Une dose quotidienne de colchicine est prescrite. Chez certains, le fait de sauter même une seule dose peut provoquer une crise. Vous et votre médecin établirez si votre enfant a besoin de colchicine et, le cas échéant, la durée du traitement.</p> <h2>Analyses en laboratoire requises chez les enfants atteints de la FMF</h2> <p>Les analyses de prélèvements sanguins effectués durant une crise révèleront normalement une inflammation très marquée. Les analyses les plus souvent demandées sont les suivantes : formule sanguine complète (FSC), vitesse de sédimentation globulaire (VSG) et dépistage de la protéine C-réactive (PCR). Les résultats sont normaux ou quasi normaux entre les crises.</p> <p>Pour déterminer s’il y a eu déclenchement d’une amyloïdose, le médecin fera un examen physique complet et commandera une analyse d'échantillons d’urine à des intervalles de six mois afin d'y déceler la présence de protéines.</p><h2>Complications of FMF</h2> <p>Without treatment, attacks of FMF will occur frequently and can be disabling for the patient. If attacks of inflammation are not controlled, there is a risk of developing amyloidosis, the most serious complication of FMF. In amyloidosis, a protein of inflammation called SAA is deposited in multiple organ systems, particularly the kidney, heart and gastrointestinal tract. Amyloidosis appears to be less common in patients born and raised in the northern hemisphere.</p>

 

 

Familial Mediterranean fever (FMF)922.000000000000Familial Mediterranean fever (FMF)Familial Mediterranean fever (FMF)FEnglishRheumatologyToddler (13-24 months);Preschooler (2-4 years);School age child (5-8 years);Pre-teen (9-12 years);Teen (13-18 years)Chest;Abdomen;Knee;Ankle;SkinImmune systemConditions and diseasesCaregivers Adult (19+)NA2015-07-15T04:00:00ZRon Laxer, MD, FRCPC;Karyl S. Barron, MD (National Institutes of Health)​​10.000000000000053.0000000000000950.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Familial Mediterranean fever (FMF) involves recurrent episodes of fever accompanied by symptoms of abdominal pain, chest pain, joint pain and/or skin rashes. Learn about causes, tests and treatments for FMF.</p><p>Familial Mediterranean fever (FMF) is a disease that involves recurrent episodes of <a href="/Article?contentid=30&language=English">fever</a> without an associated infection. Attacks of fever in FMF are usually accompanied by symptoms of inflammation in one or more sites. These may include abdominal pain, chest pain, joint pain and skin rashes, among others. It is a life-long disease and there is no known cure. Fortunately, however, it can be controlled with treatment.</p><h2>Key points</h2> <ul> <li>FMF is a disease that causes regular attacks of fever.</li> <li>The attacks are accompanied by inflammation in areas such as the abdomen, chest, joints and skin.</li> <li>FMF can be treated and controlled with a medication called colchicine.</li> </ul><h2>Symptoms of FMF</h2> <p>A typical attack of FMF starts with a sudden rise in temperature, often up to 104°F (40°C). Fever usually lasts from one to three days and in most cases is accompanied by severe abdominal pain. The pain comes from inflammation of the lining of the abdomen. Sometimes the pain is so bad that it seems as if the child has <a href="/Article?contentid=818&language=English">appendicitis</a>, even though the appendix itself is not inflamed. Severe chest pain, called pleuritis, occurs in about one-third of patients. Brief episodes of arthritis, usually involving the knee or ankle, can occur in about half of patients. About one-third of patients get painful red skin and swelling, called erysipelas. This typically occurs over the foot. These symptoms generally disappear as the fever resolves, although attacks of arthritis may last for up to one week. Rarely, arthritis affecting a single joint, including the joints of the back (sacroiliitis), may persist. Attacks may also be accompanied by severe muscle pain, called myalgia.</p><h2>How common is FMF?</h2> <p>FMF is more common in certain ethnic groups, primarily those whose ancestry was around the Mediterranean Sea. These groups include Armenians, Turks, Arabs and Jews, especially of Sephardic background. More recently, however, FMF has been diagnosed in people from many other ethnic backgrounds. Attacks of FMF begin before the age of 20 in 90% of patients. Attacks begin before age 10 in at least 50% of patients.</p> <h2>Causes of FMF</h2> <p>FMF appears to be caused by failure of the body to control the inflammation system. A protein called pyrin is an important controller of inflammation in the body. Changes (mutations) in the gene that makes pyrin, called MEFV, are found in up to 80% of FMF cases. Most cases require two mutations (one from the mother and one from the father) in the MEFV gene to result in FMF. This type of disease is called “autosomal recessive,” meaning that parents are generally carriers. There is often, but not always, a history of another family member having a similar disease. Sometimes only one or even no mutations are found in typical cases of FMF.</p><h2>Treatment of FMF</h2> <p>Fortunately, amyloidosis can almost always be prevented by stopping attacks of inflammation with the drug colchicine. Taking colchicine every day reduces the frequency of the attacks as well as the severity of the attacks that do occur, and therefore prevents the development of amyloidosis. Colchicine is prescribed as a daily medication. In some patients, missing even one dose may result in an attack. You and your doctor will decide if your child needs colchicine and if so, for how long. </p> <h2>Lab tests for children with FMF</h2> <p>During the attacks, blood tests will usually show very marked degrees of inflammation. The most commonly requested tests are a complete blood count (CBC), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). These levels return to normal or near normal between attacks.</p> <p>Your doctor will check for the development of amyloidosis by performing a general physical examination and also a urine test to check for protein in the urine every six months.</p><h2>Complications of FMF</h2> <p>Without treatment, attacks of FMF will occur frequently and can be disabling for the patient. If attacks of inflammation are not controlled, there is a risk of developing amyloidosis, the most serious complication of FMF. In amyloidosis, a protein of inflammation called SAA is deposited in multiple organ systems, particularly the kidney, heart and gastrointestinal tract. Amyloidosis appears to be less common in patients born and raised in the northern hemisphere.</p><h2>How you can help your child with FMF</h2> <p>Typical episodes of FMF should not be treated with antibiotics. However, children with FMF may also get usual childhood infections. So, if you are concerned that a fever episode is not FMF, always contact your child’s doctor.</p> <p>Also notify your child’s school about the diagnosis. Children with FMF are not contagious. They should not be excluded from school. Children with FMF should be encouraged to carry on with normal activities.</p><h2>Follow-up care at a rheumatology clinic</h2> <p>Your child may be referred to a rheumatology clinic for follow-up care. Usually, a rheumatology clinic will have a team of doctors and nurses who have a lot of experience treating children with FMF.</p> <p>Other members of the rheumatology team include:</p> <ul> <li>a social worker and a child life specialist to help with emotional and behavioural problems that might result from the disease</li> <li>a dietitian to help with your child’s diet and nutrition</li> </ul> <h3>Preparing for visits to the rheumatology clinic</h3> <p>Your child may need a blood test during a clinic visit. This will help doctors monitor the disease. Blood tests also help check for side effects of the medicine your child may be taking. A urine test will usually be needed as well. If your child needs to prepare for clinic visits in any other way, a member of the rheumatology team will tell you before the visit.</p> <p>After you have met the rheumatology team, you will know more about how to care for your child and how to plan for future clinic visits.</p>https://assets.aboutkidshealth.ca/AKHAssets/familial_mediterranean_fever.jpgFamilial Mediterranean fever (FMF)

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