Materiały źródłowe

• #1 EULAR recommendations for the management of familial Mediterranean fever | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/75/4/644

  Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. […] A set of widely accepted recommendations for the treatment and monitoring of FMF is presented, supported by the best available evidence and expert opinion. It is believed that these recommendations will be useful in guiding physicians in the care of patients with FMF. […] The ultimate goal of treatment in FMF is to obtain complete control of unprovoked attacks and minimise subclinical inflammation in between attacks. […] Biological treatment such as anti-interleukin 1 (IL-1) therapy should be considered if the inflammation cannot be controlled with adequate colchicine.

• #2 Management of familial Mediterranean fever – UpToDate

  https://www.uptodate.com/contents/management-of-familial-mediterranean-fever

  Management of familial Mediterranean fever […] GOALS OF THERAPY […] The goals of therapy for familial Mediterranean fever (FMF) are to prevent acute attacks and minimize subclinical inflammation in between attacks. Controlling inflammation even during asymptomatic intervals can prevent the development and progression of secondary (AA) amyloidosis. […] […] […] This topic will review the management of FMF.

• #3 Familial Mediterranean Fever – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560754/

  Familial Mediterranean fever (FMF) is an autoinflammatory genetic disorder that mainly affects people of Mediterranean origin. […] Early initiation with colchicine leads to improvement in the attacks and prevent amyloidosis. […] Describe the role of colchicine and other agents in the management of familial Mediterranean fever. […] Colchicine has been the treatment of choice for FMF since the 1970s. The aim of the treatment is to prevent attacks, normalize inflammation between the attacks, and prevent the development of amyloidosis. It can also halt the progression of amyloidosis. […] Colchicine works by inhibiting neutrophil chemotaxis, which is necessary for the inflammatory events in FMF. […] The treatment with colchicine is usually lifelong. Dosing varies according to age and severity of symptoms.

• #4 Pharmacological and Clinical Basis of Treatment of Familial Mediterranean Fever (FMF) with Colchicine or Analogues: An Update

  https://benthamscience.com/public/article/35772

  Familial Mediterranean Fever (FMF), an autosomal recessive disorder, is characterised by recurrent attacks of fever and serositis, lasting 24-72 hours. Since 1972 colchicine has become the drug of choice for prophylaxis against FMF attacks and amyloidosis FMF-associated. Colchicine is able to prevent activation of neutrophils, binding -tubulin and making -tubulin-colchicine complexes; this way inhibits assembly of microtubules and mitotic spindle formation; moreover its mode of action includes modulation of chemokines, prostanoids production, inhibition of neutrophil and endothelial cell adhesion molecules. The minimal daily dose in adults is 1.0 mg / die, but in children there is not a definite dose. […] FMF patients are recommended to take colchicine. Since colchicine treatment is often complicated by frequent gastrointestinal side effects, by our experience, in order to improve colchicine tolerance we recommend: lactose-free diet and treatment of intestinal bacterial overgrowth and / or Hp-infection, assessed by breath tests. […] today we are working in the design of colchicine analogues which may have lesser toxicities and a larger therapeutic window.

• #5 EULAR recommendations for the management of familial Mediterranean fever | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/75/4/644

  Treatment with colchicine should be started as soon as a clinical diagnosis is made. […] A genetic diagnosis of FMF in the absence of clinical manifestations or subclinical inflammation is not necessarily an indication to start treatment, but such patients must remain under surveillance since they may develop clinically significant disease in future, even without symptoms. […] The persistence of attacks or subclinical inflammation represents an indication to increase colchicine dose. […] Patients who continue to have one or more attacks each month despite receiving the maximally tolerated dose for at least 6 months may be considered to be a non-responder or resistant to colchicine, although compliance to treatment must be affirmed. […] AA amyloidosis is the most serious complication of uncontrolled FMF, resulting in renal failure and early death. […] Treatment for amyloidosis comprises measures to support failing organ function, including blood pressure control and dialysis for patients with renal disease.

• #6 Familial Mediterranean Fever (FMF): What It Is & Symptoms

  https://my.clevelandclinic.org/health/diseases/familial-mediterranean-fever

  Taking an oral medication called colchicine can effectively prevent or reduce these episodes. Most people with FMF have to take it daily for life. […] The standard treatment for familial Mediterranean fever is an anti-inflammatory medication called colchicine. It works by inhibiting the activity of white blood cells called neutrophils, which play a role in inflammation. Colchicine can help prevent or reduce the frequency of FMF attacks and its complications. It works for more than 90% of people with FMF. Most of these people will need to take it daily for life. […] A small number of people don’t respond to colchicine or can’t take it for health-related reasons. Alternative medications include: Anakinra, Rilonacept, Canakinumab.

• #7 Familial Mediterranean Fever Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/330284-treatment

  Colchicine is so effective in preventing attacks of familial Mediterranean fever (FMF) and preventing the development of amyloidosis that the most important aspects of medical care are to make the correct diagnosis and to institute therapy. […] Administer colchicine therapy daily in patients at risk of developing amyloidosis (eg, North African Jewish people, Turkish people, Armenian people living in Armenia). Other Sephardic Jewish people and Arabic people are at lower risk but also probably require daily colchicine therapy. […] Daily colchicine is customarily given in a dosage of 0.6 or 0.5 mg twice daily, depending on the dosage form available. However, a study in treatment-naive pediatric patients newly diagnosed with FMF found that a single 1-g daily dose was noninferior to 0.5 mg given twice daily.

• #8 Diagnosis and management of familial Mediterranean fever: Integrating medical genetics in a dedicated interdisciplinary clinic | Genetics in Medicine

  https://www.nature.com/articles/gim9201147

  Colchicine should be administered orally once the diagnosis of FMF is confirmed (or as a therapeutic trial in establishing the diagnosis). Adult dosing is 1.22.4 mg/day, whereas children usually start at 0.31.2 mg/day according to age and weight and can increase sequentially up to 2 mg/day depending on how effectively the attacks are regulated. […] It is crucial to drive home to patients (or their parents) that daily lifelong administration of colchicine is required to prevent both the fever/pain attacks and the silent amyloid deposition. […] If proteinuria is detected in a patient with FMF, more definitive renal studies are necessary, including a 24-hour urine protein assay and renal function tests (blood urea nitrogen and creatinine), and referral to a nephrologist for further work-up and management.

• #9 Familial Mediterranean Fever Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330284-guidelines

  In patients with decreased renal function, the risk of colchicine toxicity is very high, so evidence of toxicity should routinely be sought and if found, the colchicine dose should be reduced accordingly. […] Colchicine toxicity is a serious complication that should be given adequate consideration and be prevented; potential causes include exceeding the recommended dose (maximum recommended oral doses for treatment of FMF are 3 mg daily in adults and 2 mg daily in children), liver or renal failure, and concomitant administration of other drugs metabolized by cytochrome 3A4. […] If an attack is suspected, always consider other possible causes; during attacks, continue the usual dose of colchicine and use nonsteroidal anti-inflammatory drugs (NSAIDs). […] Colchicine should not be discontinued during conception, pregnancy, or lactation; current evidence does not justify amniocentesis.

• #10 Familial Mediterranean Fever Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330284-guidelines

  Ideally, FMF should be diagnosed and initially treated by a physician with experience in FMF; after diagnosis and initiation of therapy, patients can also be followed by their primary care physician in conjunction with the referral center, but if possible, patients should be seen by a physician with experience of FMF at least once per year in the long term. […] The ultimate goal of treatment in FMF is to obtain complete control of unprovoked attacks and minimize subclinical inflammation between attacks. […] Treatment with colchicine should be started as soon as a clinical diagnosis is made. […] Colchicine can be given in single or divided doses, depending on tolerance and compliance. […] Persistent attacks or subclinical inflammation represent an indication to increase the colchicine dose.

• #11 Familial Mediterranean Fever – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/hereditary-periodic-fever-syndromes/familial-mediterranean-fever

  Daily prophylactic colchicine should be initiated as soon as the diagnosis is made to prevent attacks as well as amyloidosis. Colchicine provides complete remission or distinct improvement in almost 95% of patients. If attacks or subclinical inflammation persist, the colchicine dose should be increased. Patients who are colchicine-resistant or -intolerant may be treated with IL-1 inhibitors (anakinra once a day, rilonacept weekly, or canakinumab every 4 weeks). However, the role of IL-1 inhibitors in preventing amyloidosis remains unknown, and patients taking IL-inhibitors should continue taking colchicine if tolerated. […] Widespread use of prophylactic colchicine has led to a dramatic reduction in the incidence of amyloidosis and subsequent renal failure. […] Lack of response to colchicine is often caused by poor adherence to the regimen.

• #12 Therapeutic approach to familial Mediterranean fever: a review update – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21968242/

  Familial Mediterranean fever (FMF) is a hereditary disorder characterised by recurrent attacks of fever with peritonitis or pleuritis, arthritis, myalgia or erysipelas-like skin lesions. […] Therefore, treatment of patients with FMF is beneficial not only for the prevention of the acute attacks but also for improving their prognosis. […] In conclusion, colchicine remains the mainstay therapeutic option in FMF. It is effective in various manifestations of the disease such as fever, peritonitis and pleuritis. It prevents the development of amyloidosis. It is safe in humans regarding fertility, and can be used during pregnancy and nursing. […] In the few cases where FMF is resistant to colchicine other measures, including corticosteroids, non-biological and biological DMARDs, interferon alpha and SSRIs should be employed.

• #13 About Familial Mediterranean Fever

  https://www.genome.gov/Genetic-Disorders/Familial-Mediterranean-Fever

  At present, there is no known cure for Familial Mediterranean Fever (FMF). Physicians can only treat the symptoms of the disease. […] A common therapy for FMF is daily use of the drug colchicine, a medicine that reduces inflammation. This therapy has been successful in preventing attacks of fever in 75 percent of patients who take the drug regularly, and over 90 percent of patients demonstrate a marked improvement. Even if colchicine does not prevent the fever attacks, it does prevent the amyloidosis. However, compliance in taking colchicine every day is very important. If a patient stops taking the drug, an attack can occur within a couple of days. […] Since the gene that causes FMF codes for the protein pyrin, researchers hope that by studying how this protein works they will ultimately develop improved treatments for FMF, and possibly for other conditions involving excess inflammation.

• #14 Familial Mediterranean Fever

  https://www.printo.it/pediatric-rheumatology/IE/info/20/Familial-Mediterranean-Fever

  Familial Mediterranean Fever (FMF) is a genetically transmitted disease. Patients suffer from recurrent bouts of fever, accompanied by abdominal or chest pain, joint pains and swelling. […] FMF cannot be cured but it can be treated with life-long use of colchicine. In this way, recurrent attacks can be prevented or decreased and amyloidosis can be prevented. If the patient stops taking the drug, the attacks and the risk of amyloidosis will recur. […] The treatment for FMF is simple, inexpensive and does not involve any major drug side effects, as long as it is taken in the right dose. Colchicine is the drug of choice for the treatment of FMF. After the diagnosis is made, the child must take the drug for the rest of his/her life. If taken properly (every day, at sufficient dose) the attacks: disappear in about 60% of patients; a partial response is obtained in 30%; and it is ineffective in 5-10% of patients. This treatment not only controls the attacks but also eliminates the risk of amyloidosis.

• #15 Familial Mediterranean Fever Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330284-guidelines

  Compliant patients who continue to have one or more attacks each month despite receiving the maximally tolerated dose of colchicine for at least 6 months can be considered nonresponders or resistant; alternative biological treatments are indicated in these patients; colchicine should be coadministered with biological therapies, as it may reduce the risk of amyloidosis despite persistence of attacks. […] FMF treatment needs to be intensified in AA amyloidosis, using the maximal tolerated dose of colchicine and supplemented with biologics as required. […] Periods of physical or emotional stress can trigger FMF attacks, and it may be worth temporarily increasing the dose of colchicine. […] Response, toxicity, and compliance should be monitored every 6 months. […] Liver enzymes should be monitored regularly in patients with FMF treated with colchicine; if elevations greater than twofold the upper limit of normal occur, the colchicine dose should be reduced and the cause further investigated.

• #16 Familial Mediterranean Fever

  https://www.printo.it/pediatric-rheumatology/IE/info/20/Familial-Mediterranean-Fever

  It is not easy to accept that a child must take pills forever. Parents are often worried about the potential side effects of colchicine. It is a safe drug with minor side effects that usually respond to dose reduction. The most frequent side effect is diarrhoea. […] FMF requires life-long preventive treatment. […] No complementary therapy is known for FMF. […] Children being treated should have blood and urine tests at least twice a year. […] If treated properly with life-long colchicine, children with FMF live a normal life. If there is a delay in diagnosis or lack of compliance with treatment, the risk of developing amyloidosis increases, which results in a poor prognosis. […] No, because it is a genetic disease. However, life-long therapy with colchicine gives the patient the opportunity to live a normal life, without restrictions and with no risk of developing amyloidosis.

• #17 Familial Mediterranean fever – Wikipedia

  https://en.wikipedia.org/wiki/Familial_Mediterranean_fever

  Attacks are self-limiting, and require analgesia and NSAIDs (such as diclofenac). Colchicine, a drug otherwise mainly used in gout, decreases attack frequency in FMF patients. The exact way in which colchicine suppresses attacks is unclear. While this agent is not without side effects (such as abdominal pain and muscle pains), it may markedly improve the quality of life in patients. The dosage is typically 12 mg a day. Development of amyloidosis is delayed with colchicine treatment. Interferon is being studied as a therapeutic modality. […] Approximately 5-10% of FMF cases are resistant to colchicine therapy alone. In these cases, adding anakinra to the daily colchicine regimen has been successful. Canakinumab, an anti-interleukin-1-beta monoclonal antibody, has likewise been shown to be effective in controlling and preventing flare-ups in patients with colchicine-resistant FMF and in two additional autoinflammatory recurrent fever syndromes: mevalonate kinase deficiency (hyper-immunoglobulin D syndrome, or HIDS) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS).

• #18 Familial Mediterranean fever

  https://dermnetnz.org/topics/familial-mediterranean-fever

  The most common reason for failure to respond to colchicine is poor compliance due to gastrointestinal upset. However, 5-10% do not respond to colchicine and this may be due to ABCB1 gene polymorphisms affecting colchicine uptake by mononuclear cells. […] Thalidomide and biologic agents, such as daily subcutaneous anakinra (an interleukin-1 receptor antagonist) or etanercept, may then be considered to prevent attacks and the development of amyloidosis. To reduce the severity of an attack, single-dose methylprednisolone or anakinra at the start of episodes have been reported to relieve symptoms.

• #19 Familial Mediterranean Fever – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560754/

  Patients with a more complicated disease such as frequent attacks or presence of amyloidosis can be given higher doses (2 to 2.5mg) as tolerated by the patients and if the liver and kidney function are normal. […] Compliance with the treatment must be addressed strongly to the patients since it is a common cause of lack of response to the therapy. […] If patients are still not responding to the treatment, then the dose of colchicine should be increased. […] IL-1 inhibitors are the second-line drugs for the patients who have colchicine resistant FMF or who have an intolerance to colchicine. […] Other drugs […] Tumor necrosis factor (TNF)-alpha inhibitors thalidomide, infliximab, and etanercept (a biologic TNF inhibitor that binds both TNF-alpha and TNF-beta) have also been described in some cases, but the real efficacy is still not established. […] Colchicine prevents the development of amyloidosis, so patients who have never been on colchicine or patients who present late in the disease process have higher chances of developing amyloidosis.

• #20 Frontiers | Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2017.00253/full

  Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease (AID) affecting mainly the ethnic groups originating from Mediterranean basin. […] Recently, an international group of experts has published the recommendations for the management of FMF. Colchicine is the mainstay of FMF treatment, and its regular use prevents attacks and controls subclinical inflammation in the majority of patients. […] Colchicine is the mainstay of FMF treatment, and its regular use prevents attacks and suppresses chronic subclinical inflammation. […] Anti-IL-1 drugs emerged as promising treatment options in patients who fail to response or tolerate colchicine. […] The EULAR recommendations emphasize that the aim of FMF treatment is obtaining the control of acute attacks, minimizing the chronic and subclinical inflammation, preventing complications, and providing an acceptable quality of life.

• #21 Familial Mediterranean fever // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/familial-mediterranean-fever

  There’s no cure for familial Mediterranean fever. However, treatment can help relieve symptoms, prevent attacks and prevent complications caused by inflammation. […] Medications used to relieve symptoms and prevent attacks of FMF include: […] Colchicine (Colcrys), taken in pill form, reduces inflammation in your body and helps prevent attacks and the development of amyloidosis. […] For people whose signs and symptoms aren’t controlled with colchicine, medications that block a protein called interleukin-1, which is involved in inflammation, may be prescribed. Canakinumab (Ilaris) is approved by the U.S. Food and Drug Administration (FDA) for FMF. […] Colchicine is effective in preventing attacks for most people. To lessen the severity of symptoms during an attack, your health care provider may recommend intravenous fluids and medications to reduce fever and inflammation and control pain. […] Regular appointments with your health care provider are important to monitor your medications and your health.

• #22 Rare Familial Mediterranean Fever

  https://www.webmd.com/arthritis/familial-mediterranean-fever

  Theres no cure for FMF, but treatments can prevent and relieve symptoms. […] Colchicine is a generic medicine that controls inflammation in FMF. Its taken as a pill once or twice a day. Colchicine can prevent FMF flares, but if you or your child is already having an episode, the drug wont treat it. Colchicine needs to be taken regularly to work. Flares can return if you miss even one dose. Children who take colchicine regularly can have a full, normal life with FMF. […] If colchicine doesnt work or your child cant tolerate it, drugs that block interleukin-1, a protein linked to inflammation in FMF, may help. These drugs include anakinra (Kineret), canakinumab (Ilaris), and rilonacept (Arcalyst). […] Nonsteroidal anti-inflammatory drugs (NSAIDs) and pain medications, or analgesics, may help relieve fevers or joint pain during a flare. […] With treatment, you can have a normal, full life with FMF.

• #23 Familial Mediterranean Fever Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/330284-treatment

  Patients who have an inadequate response to colchicine but who cannot afford biologic agents may benefit from the addition of dapsone to colchicine. […] In patients whose conditions do not respond to colchicine, biologic therapy in particular, agents directed toward interleukin-1 (IL-1) may be effective. […] Rilonacept, given by once-weekly subcutaneous injection, has been shown, in combination with continuation of colchicine, to reduce the number of attacks in patients who did not respond optimally. […] A systematic review found that in patients who do not respond to colchicine or cannot tolerate it, anti-IL-1 treatment resulted in a complete response to therapy, without a single attack during treatment, in 76.5% of patients treated with anakinra and 67.5% of those treated with canakinumab.

• #24 Anti-Interleukin-1 Treatments are Effective in Familial Mediterranean Fever – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/news/anti-il-1-treatment-effective-for-fmf/

  Anti-interleukin-1 agents are safe and effective treatment options among patients with familial Mediterranean fever. […] Treatments aim to suppress recurrent attacks and ongoing inflammation. […] Our meta-analysis showed that anti-IL-1 agents provided significant improvement in laboratory outcomes of FMF patients by reducing ESR and the level of CRP. […] Pooled data demonstrated that 60% (95% CI, 49%-72%) of adult patients and 81% (95% CI, 72%-89%) of pediatric patients achieved complete remission of attacks with anti-IL-1 treatment. […] A total of 93% (95% CI, 87%-99%) of adult patients and 91% (95% CI, 80%-103%) of pediatric patients had a more than 50% reduction in attack frequency. […] C-reactive protein (CRP) levels decreased after treatment with anti-IL-1 agents by 36.56 mg/L (95% CI, -52.96 mg/L to -20.16 mg/L) among adult patients and 50.6 mg/L (95% CI, -70.94 mg/L to -30.25 mg/L) among pediatric patients.

• #25 Anti-Interleukin-1 Treatments are Effective in Familial Mediterranean Fever – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/news/anti-il-1-treatment-effective-for-fmf/

  Erythrocyte sedimentation rates (ESR) were decreased by 30.36 mm/h (95% CI, -43.44 mm/h to -17.29 mm/h) among adult patients and -37.05 mm/h (95% CI, -49.8 mm/h to -24.29 mm/h) among pediatric patients after anti-IL-1 treatment. […] Disease activity scores were decreased among adult patients following anti-IL-1 treatment (standardized mean difference, -2.17; 95% CI, -3.15 to -1.19). […] One in 4 adult patients (25%; 95% CI, 13%-37%) and about one in 10 pediatric patients (12%; 95% CI, 3%-21%) experienced a minimum of 1 AE during anti-IL-1 treatment. […] The study authors concluded, Our meta-analysis showed that anti-IL-1 agents provided significant improvement in laboratory outcomes of FMF patients by reducing ESR and the level of CRP.

• #26 Familial Mediterranean Fever Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/330284-treatment

  After colchicine has been started, EULAR recommends following patients closely for 36 months to observe the therapeutic effect. In patients who do not respond to twice-a-day dosing, administer colchicine three, or even four, times a day. […] In patients whose conditions were not responsive to oral colchicine, the addition of intravenous (IV) dosing 1 mg IV once a week reduced the number of attacks in 10 of 13 patients and the severity of attacks in 6 of 13 patients. […] Colchicine also stabilizes the amount of proteinuria in patients with amyloid nephropathy. […] A Turkish study found that in children who were heterozygous for MEFV variants and required initiation of colchicine treatment after experiencing symptoms of FMF, colchicine may be successfully discontinued in some cases, if very careful follow-up is provided.

• #27 Canakinumab Treatment Effective for Familial Mediterranean Fever – The Rheumatologist

  https://www.the-rheumatologist.org/article/canakinumab-treatment-effective-familial-mediterranean-fever/

  For patients with established type amyloid A amyloidosis, anti-IL-1 treatment can even reverse proteinuria. […] Now, Tolga Yildirim, MD, PhD, associate professor of nephrology at Hacettepe University in Turkey, and colleagues present a case series that suggests that canakinumab is a useful first-line option for the treatment of renal transplant recipients with FMF. […] Their paper, published online Feb. 14 in the Journal of Nephrology, indicates that canakinumab has equal efficacy and better tolerability than anakinra in these patients. […] The authors report their three patients achieved a complete clinical response, including the elimination of attacks and normalization of serum C-reactive protein (CRP) levels. These benefits were achieved without significant side effects.

• #28 EULAR recommendations for the management of familial Mediterranean fever | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/75/4/644

  Periods of physical or emotional stress can trigger FMF attacks, and it may be worth temporarily increasing the dose of colchicine. […] There is neither agreement on the definition of adequate response nor consensus on the number of attacks per year that may be considered acceptable; this tolerability level should be judged in the context of the quality of life of the patient. […] Colchicine should not be discontinued during conception, pregnancy or lactation; current evidence does not justify amniocentesis. […] In general, men need not stop colchicine prior to conception; in the rare case of azoospermia or oligospermia proven to be related to colchicine, temporary dose reduction or discontinuation may be required. […] Chronic arthritis in a patient with FMF might need additional medications, such as disease modifying antirheumatic drugs (DMARDs), intra-articular steroid injections or biologics. […] If a patient is stable with no attacks for more than 5 years and no elevated APR, dose reduction could be considered after expert consultation and with continued monitoring.

• #29 Familial Mediterranean fever: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000363.htm

  The goal of treatment for FMF is to regulate 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. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to treat fever and pain.

• #30 Familial Mediterranean Fever – Children’s Health Issues – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/children-s-health-issues/hereditary-periodic-fever-syndromes/familial-mediterranean-fever

  Colchicine is taken to reduce or eliminate the number of painful attacks and eliminate the risk of kidney failure due to amyloidosis. […] Taking colchicine daily by mouth eliminates or greatly reduces the number of painful attacks in about 95% of people. Also, colchicine greatly reduces the risk of kidney failure due to amyloidosis. In pregnant women, colchicine helps prevent attacks that might lead to a miscarriage. […] If colchicine stops working or people are not able to tolerate it, other medications such as canakinumab, anakinra, or rilonacept, injected under the skin, may help. These medications modify how the immune system functions and thus help reduce inflammation. […] Ibuprofen or acetaminophen can be given to relieve pain and fever.

• #31 Familial Mediterranean Fever Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330284-guidelines

  In general, men need not stop colchicine prior to conception; in the rare case of azoospermia or oligospermia proven to be related to colchicine, temporary dose reduction or discontinuation may be required. […] Chronic arthritis in a patient with FMF might need additional medications, such as disease-modifying antirheumatic drugs (DMARDs), intra-articular steroid injections, or biologics. […] In protracted febrile myalgia, glucocorticoids lead to the resolution of symptoms; NSAID and interleukin-1 blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain. […] If a patient is stable, with no attacks for more than 5 years and no elevation in acute phase reactants (eg, serum amyloid A protein, C-reactive protein), dose reduction could be considered after expert consultation and with continued monitoring.

• #32 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20250423/New-recommendations-for-the-treatment-of-familial-Mediterranean-fever.aspx

  In 2016, EULAR – The European Alliance of Associations for Rheumatology – published recommendations on the treatment of familial Mediterranean fever (FMF). […] The new work, published in the April 2025 issue of the Annals of the Rheumatic Diseases, includes 12 individual recommendations and four overarching principles. […] They also stress that the treatment goal is to achieve at least minimal activity and control of subclinical inflammation to prevent damage – and that this will require lifetime management and prophylaxis. […] The 12 new recommendations focus on key management steps, from first-line treatment with colchicine and dosing considerations through to second-line options and the biologic therapies that may be considered. […] EULAR and PReS are confident that this revised set of recommendations will support rheumatologists and other healthcare professionals in managing FMF in patients of all ages.

• #33 Treat-to-target strategies for the management of familial Mediterranean Fever in children | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-023-00875-y

  The treat-to-target (T2T) concept introduces treatment escalation and de-escalation depending on the achievement of numerical therapeutic targets. […] The aim of this initiative was to develop a T2T approach for the treatment of FMF that (i) unifies the plethora of available disease activity scores in the form of a multidimensional treatment target and (ii) takes into account the recent emergence of novel therapeutic agents. […] The proposed consensus treatment plan for the management of FMF incorporates multidimensional targets allowing transparent treatment decisions, which will promote personalised disease management and increase adherence to therapy. […] The developed consensus statements together with the level of agreement as well as the level of evidence and the grade of recommendation are summarised in Table 1.

• #34 Familial Mediterranean fever (FMF) | Sobi

  https://www.sobi.com/en/familial-mediterranean-fever-fmf

  Early diagnosis and treatment can help most people with FMF live normal lives. […] Leading a healthy lifestyle with a good diet, exercise, weight control, and stress relief can help people manage pain and stay healthy. […] Medications exist that in many cases not only manage but actually prevent symptoms. […] By consulting healthcare professionals who specialise in FMF, such as rheumatologists or geneticists, people with FMF can receive expert guidance and personalised treatment options.

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