Materiały źródłowe

• #1 Management of familial Mediterranean fever – UpToDate

  https://www.uptodate.com/contents/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.

• #2 Management of familial Mediterranean fever – UpToDate

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

  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.

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

  https://www.eurekaselect.com/90796/article

  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. […] According to current guidelines and because of rare side effects of the drug, FMF patients are recommended to take colchicine. […] 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.

• #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. […] The minimal daily dose in adults is 1.0 mg / die, but in children there is not a definite dose. […] According to current guidelines and because of rare side effects of the drug, FMF patients are recommended to take colchicine. […] 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. […] Since our data showed that 10-15% of FMF patients seem are non-responders or intolerant to colchicine, today we are working in the design of colchicine analogues which may have lesser toxicities and a larger therapeutic window.

• #5 Colchicine prophylaxis in familial Mediterranean fever: reappraisal after 15 years – PubMed

  https://pubmed.ncbi.nlm.nih.gov/2042056/

  As determined in this study of 45 patients, the prolonged use of colchicine therapy in familial Mediterranean fever (FMF) is safe and effective in preventing flares of FMF and amyloidosis. […] Its discontinuation predisposes patients to acute FMF attacks and the development of amyloidosis.

• #6 Long-term colchicine treatment in children with familial Mediterranean fever – PubMed

  https://pubmed.ncbi.nlm.nih.gov/1859491/

  Three hundred fifty children (younger than age 16) who had familial Mediterranean fever (FMF) were given continuous prophylactic treatment with colchicine (1-2 mg/day) for 6-13 years. Complete remission of febrile attacks was achieved in 64% of the patients, and partial remission in 31%. […] None of the children developed amyloidosis while on the colchicine regimen. The efficacy of long-term colchicine treatment of children with FMF makes early diagnosis life saving.

• #7 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 starting dose of 0.5mg/day (0.6mg/day in case tablets contain 0.6mg) for children 5years of age, 0.51.0mg/day (1.2mg/day in case tablets contain 0.6mg) for children 510years of age, 1.01.5mg/day (1.8mg/day in case tablets contain 0.6mg) in children 10years of age and in adults is recommended. […] The persistence of attacks or subclinical inflammation represents an indication to increase colchicine dose. […] If inflammation persists despite adherence to the advised initial dose of colchicine, as defined by continuing attacks or elevated APR between attacks, colchicine dose may be increased by 0.5mg/day (or 0.6mg/day depending on the available drug formulation) with careful monitoring of side effects.

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

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

  Response, toxicity and compliance should be monitored every 6months. […] Compliance with colchicine taken on a regular daily basis is the cornerstone of management of FMF, with potential to reduce the frequency and severity of clinical attacks and prevent the development of AA amyloidosis, renal failure and premature death in most patients. […] Liver enzymes should be monitored regularly in patients with FMF treated with colchicine; if liver enzymes are elevated greater than twofold the upper limit of normal, colchicine should be reduced and the cause further investigated. […] In a minority of cases, discontinuation of colchicine will lead to normalisation of the enzyme values, but in most there proves to be no clear association with the drug. […] In patients with decreased renal function, the risk of colchicine toxicity is very high and therefore evidence of toxicity should routinely be sought and the colchicine dose reduced accordingly. […] There is no specific cut-off for reduced glomerular filtration rate to guide colchicine dose reduction, but it has been shown that patients with FMF on colchicine with elevated creatinine are prone to develop muscle pain with myopathy.

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

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

  Colchicine toxicity is a serious complication that should be given adequate consideration and be prevented. […] Conditions that may lead to colchicine toxicity are as follows: Exceeding the recommended dose, liver or renal failure, and concomitant administration of other drugs. […] Colchicine should not be discontinued during conception, pregnancy or lactation; current evidence does not justify amniocentesis. […] Discontinuation of colchicine may lead to exacerbation of FMF attacks and in long term to the development of amyloidosis. […] Chronic arthritis in a patient with FMF might need additional medications, such as disease modifying antirheumatic drugs (DMARDs), intra-articular steroid injections or biologics. […] Approximately 5% of patients with FMF develop chronic joint involvement, the majority resembling spondyloarthritis with sacroiliitis and peripheral monoarthritis or oligoarthritis and rarely resembling juvenile idiopathic arthritis.

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

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

  Compliant patients not responding to the maximum tolerated dose of colchicine can be considered non-responders or resistant; alternative biological treatments are indicated in these patients. […] Evidence for therapeutic options for patients resistant or intolerant to colchicine is limited, but case reports and case series have suggested that IL-1 blockade is a promising second-line therapy. […] FMF treatment needs to be intensified in AA amyloidosis using the maximal tolerated dose of colchicine and supplemented with biologics as required. […] The development of amyloidosis can be prevented in most patients by suppressing chronic inflammatory activity with the measures already discussed. […] Periods of physical or emotional stress can trigger FMF attacks, and it may be worth temporarily increasing the dose of colchicine. […] Recognised trigger factors include physical or emotional stress, menstruation, physical trauma, exposure to cold, infections, inflammation, high fat intake, starvation, sleeplessness and tiredness.

• #8 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

  It is suggested that colchicine should be started as soon as the patient is clinically diagnosed as having FMF. […] The optimal dosage of colchicine varies between studies and different clinical practices. […] Colchicine treatment is lifelong in FMF. […] Compliance with colchicine is very important for proper management of FMF. […] Anti-IL-1 therapy seems to be a promising second-line therapy in refractory or intolerant patients. […] Besides IL-1 blockade, FMF patients with chronic arthritis and/or sacroiliitis could benefit from disease-modifying antirheumatic drugs or anti-TNF agents.

• #9 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. […] 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.

• #10 Familial Mediterranean fever – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/familial-mediterranean-fever/diagnosis-treatment/drc-20372475

  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. […] Colchicine is effective in preventing attacks for most people. […] Regular appointments with your health care provider are important to monitor your medications and your health.

• #11 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. […] 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.

• #12 Pregnancy Outcome in Women With Familial Mediterranean Fever: A Retrospective Analysis of 50 Cases With a 10-Year Experience | Volume 29 – Issue 2 – June 2014 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/567

  A total of 42 patients (84%) were on drug therapy of colchicine. […] The course of the pregnancy was free of venous complications with low molecular weight heparin prophylaxis. […] A favorable pregnancy and perinatal outcome may be expected in patients with FMF treated with colchicine. […] However, close followup with prenatal test and laboratory investigations for maternal and fetal well-being including hypercoagulation disorders are thought to be essential. […] In our study population, the courses of pregnancies were variable, all were attack-free but one had cerebrovascular accident as a result of FMF-related vasculitis and the other needed daily hemodialysis (and sepsis) due amyloidosis-related end stage renal disease. […] Under such conditions, we recommend administration of low molecular weight heparin at prophylactic dosage during pregnancy.

• #13 Pregnancy Outcome in Women With Familial Mediterranean Fever: A Retrospective Analysis of 50 Cases With a 10-Year Experience | Volume 29 – Issue 2 – June 2014 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/567

  In conclusion, although our study population is small, favorable pregnancy and perinatal outcomes can be expected in patients with FMF treated with colchicine. […] Due to the subclinical inflammation leading to a hypercoagulable state, we recommend searching for thrombophilia and giving appropriate anticoagulant prophylaxis during the course of pregnancy. […] Furthermore, close follow-up with prenatal test and laboratory investigations for maternal and fetal well-being are suggested to be essential.

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

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

  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. […] 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. […] 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.

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

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

  Canakinumab was approved by the US Food and Drug Administration for FMF in 2016. […] A systematic review of the literature on canakinumab found that even though most patients included in the studies had a particularly severe phenotype, nearly 80% of patients did not experience a single attack after the initiation of therapy, and almost all the remaining patients had significantly reduced disease activity. […] High-fat and high-salt foods, and possibly wheat, have been suggested as triggers of FMF attacks, but research on this association has yielded conflicting results. It is possible that a diet rich in antioxidants and anti-inflammatory supplements could partially reduce symptoms and improve well-being in patients with FMF.

• #16 Patients with Familial Mediterranean Fever (FMF) Under Canakinumab Treatment – Long-term Efficacy and Safety Interim Data of the RELIANCE Registry – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/patients-with-familial-mediterranean-fever-fmf-under-canakinumab-treatment-long-term-efficacy-and-safety-interim-data-of-the-reliance-registry/

  Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and serositis, with a risk of severe complications (e. g. amyloidosis). Treatment of FMF according to EULAR recommendations aims to control acute relapses and subclinical inflammation and improve patients quality of life. Clinical data suggest that inhibition of interleukin-1 with canakinumab (CAN) is effective in controlling and preventing relapses in FMF patients. […] Interim data from FMF patients in the RELIANCE study, the longest running canakinumab registry, confirm the efficacy and safety of long-term treatment with canakinumab. Dose adjustments during the course of therapy are possible.

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

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

  Familial Mediterranean fever (FMF) is, perhaps, the oldest and most common hereditary autoinflammatory disease. Colchicine is the first-line treatment for FMF because, for most patients, it prevents both inflammatory attacks and the development of amyloidosis. […] Unfortunately, canakinumab, a human immunoglobulin (Ig) G antibody directed against IL-1 beta, has not been shown to prevent the development of amyloidosis. […] 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.

• #18 A Case with Familial Mediterranean Fever which Responds Well to Adalimumab Treatment | Volume 26 – Issue 2 – June 2011 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/407

  In the treatment of FMF, colchicine is commonly used because of its potent inhibitory effect on neutrophile chemotaxis. Nearly one-fourth of FMF patients are partially or completely resistant to colchicine. Tumor necrosis factor alpha inhibitors have been reported to be effective in the treatment of FMF in many case reports. Most of the patients were treated with infliximab, and one patient was treated with etanercept. Our patient responded to TNF- (adalimumab) treatment. After adalimumab was added to his treatment regimen with colchicine, a marked decrease was observed in FMF attacks, and AS disease activity improved. […] In FMF patients who do not respond to colchicine, TNF- inhibitors may be considered a treatment option.

• #19

  https://link.springer.com/article/10.1007/s11739-024-03576-w

  Several possible factors are hypothesized to trigger familial Mediterranean fever (FMF) attacks; however, there is no consensus on this matter. […] Identifying trigger factors can lead to better preventive measures and personalized therapies to decrease familial Mediterranean fever attacks. Patients can significantly decrease the number of familial Mediterranean fever attacks they experience by managing psychological stress and avoiding physical factors such as cold exposure and fatigue. […] The prophylactic use of colchicine prevents FMF attacks and amyloidosis development. […] Knowing the triggering factors of FMF attacks can help predict an upcoming attack and take preventative actions. […] In conclusion, this study shows the high prevalence of triggering factors among patients with FMF. Advancements in this area have the potential to alter the way we manage patients with FMF, as it can provide new ways to prevent FMF attacks. This can result in increased implementation of preventive measures in the management of FMF. Psychological stress and fatigue were the most common triggers of FMF attacks among our patients. Patients can significantly decrease the number of FMF attacks they experience by managing psychological stress and avoiding physical factors such as cold exposure and fatigue. Determining trigger factors and their relationship with patient-specific factors such as mutation status and ethnicity can result in personalized treatment strategies for the prevention of FMF attacks. Developments in this area will lead to a decrease in the number of attacks and improved disease control.

• #20

  https://npistanbul.com/en/what-is-familial-mediterranean-fever-fmf

  Treatment of Familial Mediterranean Fever (FMF) focuses on relieving symptoms and preventing attacks. Colchicine is the main drug for FMF and controls attacks by reducing inflammation. In some cases, additional anti-inflammatory or immunosuppressive drugs may be used. […] Regular monitoring for the risk of amyloidosis and additional treatments if necessary. A healthy lifestyle is part of the treatment and regular exercise, a balanced diet and stress avoidance support the overall health of patients. […] Treatment plans are individualized and tailored to the patient’s symptoms and health status. Patients should stay in regular contact with their doctor and follow their treatment plan. […] Special circumstances, such as pregnancy planning, may require a review of the treatment plan. This holistic treatment approach aims to improve the quality of life of FMF patients.

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

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

  A study by Ozcakar et al. in 2010 concluded that apart from preventing acute episodes, colchicine also significantly improved height development and weight parameters in children with FMF. […] 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. […] Although cytogeneticists are familiar with colchicine’s dramatic effect on cultured cells, patients (and parents) can be reassured that it is a very safe drug in vivo, at least in the doses used to treat FMF. […] The treatment of FMF does not entail expensive, difficult, or esoteric enzyme replacement, recombinant gene therapy, specialized diet, or synthesized small molecular ligand inhibitor treatment, but rather a common, generic drug that has been around for centuries.

• #22 Colchicine treatment can be discontinued in a selected group of pediatric FMF patients | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-022-00780-w

  Familial Mediterranean Fever (FMF) patients are required to adhere to a life-long treatment with colchicine, primarily for preventing amyloidosis. […] The mainstay of FMF treatment is colchicine, which reduces the frequency as well as the intensity of disease exacerbations. Colchicine also alleviates the inflammatory response and prevents progression to amyloidosis. […] While colchicine is relatively well tolerated, it can cause adverse reactions and toxicity. Moreover, patients may experience a life-long treatment as a burden. […] In recent years, few studies showed colchicine-free remission in FMF patients but there are no detailed recommendations to whom colchicine cessation can be tried. […] This study supports the concept of colchicine free remission in a minority of FMF patients (3%). Holding treatment, under close monitoring, may be reasonable when selecting the appropriate patients.

• #23 Colchicine treatment can be discontinued in a selected group of pediatric FMF patients | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-022-00780-w

  It is important to emphasize that a close clinical and laboratory follow up is required following treatment cessation. We recommend following those patients every 4 months with a careful assessment for any clinical symptom of FMF (such as unexplained fever, serositis, ELE), elevated inflammatory markers (ESR, CRP, SAA) or proteinuria. If all signs and symptoms are negative, then continuing with a close follow up off-colchicine is reasonable. Importantly, in group 2 we re-initiate colchicine safely following close monitoring due to clinical or laboratory features of active disease. All responded well and none had proteinuria. […] This study supports the concept of colchicine free remission (remission while not taking colchicine) in a minority of FMF patients (3%). Holding treatment may be reasonable when selecting the appropriate patients and under close monitoring.

• #24 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 objective of this initiative was to develop a treat-to-target (T2T) approach for the management of patients with Familial Mediterranean Fever (FMF), including the definition of a complex treatment target, and establish strategies that improve patient care and long-term outcome. […] Colchicine represents the mainstay of FMF therapy and its efficacy in preventing attacks and the occurrence of amyloidosis has been shown in clinical trials and large cohort studies. The continuous life-long colchicine application resolves symptoms in up to 2/3 of patients, in up to 1/3 of patients this therapy significantly improves clinical symptoms and systemic inflammation. […] The treat-to-target (T2T) concept introduces treatment escalation and de-escalation depending on the achievement of numerical therapeutic targets.

• #25 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 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.

• #26 Familial Mediterranean Fever

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

  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. […] 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.

• #26 Familial Mediterranean Fever

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

  Familial Mediterranean Fever requires life-long preventive treatment. […] 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. […] 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. […] 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. […] Therefore, it is crucial for the doctors to repeatedly explain to parents and the patient how vital it is to take this drug in the dose prescribed. […] FMF requires life-long preventive treatment.

• #27 2024 Management of Familial Mediterranean Fever Recommendations | RheumNow

  https://rheumnow.com/news/2024-management-familial-mediterranean-fever-recommendations

  FMF requires lifetime management, including long-term prophylaxis with colchicine; therefore, treatment adherence is the cornerstone of FMF management. […] Treatment with colchicine should start as soon as a clinical diagnosis is made. […] Colchicine should be continued during conception, pregnancy, and lactation; amniocentesis is not currently recommended.

• #28 Factors Affecting Colchicine Adherence in Pediatric Familial Mediterranean Fever – The Journal of Pediatric Research

  https://www.jpedres.org/articles/factors-affecting-colchicine-adherence-in-pediatric-familial-mediterranean-fever/doi/jpr.galenos.2023.34437

  Patients with FMF should be questioned about their medication adherence at every visit, and children with a high risk of colchicine non-adherence should be followed up more closely. […] Adolescent patients, children with severe disease and frequent relapses, those treated with higher doses of colchicine, those with M694V homozygosity, and those with homozygous M694V mutations were observed to have a higher risk of colchicine non-adherence. […] Colchicine is defined as the best treatment option for reducing attacks and preventing complications of FMF, such as amyloidosis. […] It is crucial to predict the factors affecting colchicine adherence in the management of patients with FMF.

• #29 Familial Mediterranean Fever | Rare Diseases Center

  https://rarediseases.ucla.edu/clinics/familial-mediterranean-fever

  Colchicine, a drug used to treat gout (a form of arthritis), is the first-line treatment. It prevents these disabling attacks in more than 90% of patients and prevents amyloidosis. […] Besides diagnosis and treatment of FMF, another primary goal of the clinic is the education of medical students, residents, and fellows; patients and their families; and the medical and lay communities at large.

• #30 Familial Mediterranean fever: kids & teens | Raising Children Network

  https://raisingchildren.net.au/guides/a-z-health-reference/familial-mediterranean-fever

  Familial Mediterranean fever is a genetic condition that causes fevers and inflammation in various parts of the body. […] If your child has familial Mediterranean fever, theyll usually need to take colchicine every day. This can prevent severe attacks and long-term complications of familial Mediterranean fever for example, kidney problems. […] A key part of managing your childs familial Mediterranean fever is making sure your child regularly takes their colchicine and attends all doctors appointments for check-ups.

• #31 Familial Mediterranean Fever

  https://rheumatology.org/patients/familial-mediterranean-fever

  Lifelong treatment to control inflammation may prevent injury to organs. […] To prevent FMF symptoms, take colchicine as prescribed without skipping doses. […] With regular treatment, children with FMF can stay active, pursue their education and lead productive adult lives.

• #32 Familial Mediterranean Fever: Practice Essentials, Pathophysiology, Etiology

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

  Colchicine is extremely effective in preventing attacks of FMF and preventing the development of amyloidosis. Colchicine is typically administered daily, but if attacks are rare and patients can determine when they are beginning, intermittent treatment at the onset of attacks may be sufficient. An important benefit of treatment is the prevention of secondary amyloidosis, which otherwise could result in kidney failure. […] Patients with FMF need to understand the importance of strict compliance with daily colchicine therapy.

• #33 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. […] Widespread use of prophylactic colchicine has led to a dramatic reduction in the incidence of amyloidosis and subsequent renal failure. […] 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.

• #34 Familial Mediterranean fever | EBSCO Research Starters

  https://www.ebsco.com/research-starters/health-and-medicine/familial-mediterranean-fever

  Those at higher risk include Sephardic Jews, North African Arabs, Armenians, Turks, Greeks, and Italians, although cases have also been documented in individuals without a Mediterranean background. […] While there is no cure for FMF, daily administration of colchicine can significantly reduce the frequency and severity of attacks, and it is also effective in preventing complications such as amyloidosis, which can lead to renal failure. […] There is no way to prevent FMF. Widespread use of prophylactic colchicine has led to a dramatic reduction in attacks and complications.

• #35 Frontiers | Familial Mediterranean fever in Romania: a case report and literature review

  https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2024.1546387/full

  Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent fever and systemic inflammation, most prevalent in Eastern Mediterranean populations. […] Genetic testing aids diagnosis in non-endemic regions, enabling early colchicine treatment to prevent complications. […] The first-line treatment for familial Mediterranean fever is colchicine. […] Lifetime colchicine prophylaxis is recommended for all patients, regardless of symptoms, unless a severe side effect occurs. […] In patients who consistently adhere to colchicine therapy, the long-term risk of amyloidosis is less than 1%, even if attacks are not completely controlled. […] The objectives in treating FMF include enhancing quality of life, decreasing the frequency, severity, and duration of attacks, and preventing long-term complications, especially AA amyloidosis, by reducing chronic and subclinical inflammation. […] Additional treatment is available for patients with colchicine resistance or incomplete response. […] FMF has an excellent prognosis if diagnosed and treated early, before complications arise.

• #36 Familial Mediterranean fever: current perspectives | JIR

  https://www.dovepress.com/familial-mediterranean-fever-current-perspectives-peer-reviewed-fulltext-article-JIR

  Colchicine remains the principle therapy, and the aim of treatment is to prevent acute attacks and the consequences of chronic inflammation. […] Colchicine still constitutes the mainstay of FMF treatment, and the aim of the treatment should be preventing acute attacks and amyloidosis, decreasing the chronic inflammation, and providing an acceptable quality of life. […] Colchicine is still the main form of treatment for patients with FMF. Since 1972, colchicine has been used for treatment in FMF patients. It decreases attack frequency and increases the quality of life. Furthermore, Livneh et al have shown that colchicine can effectively prevent the complication of secondary amyloidosis in FMF patients. […] The treatment recommendations are about colchicine dosage, maximum dosage of colchicine in children and adults, definition of colchicine resistance, and treatment alternatives in colchicine-resistant patients. Colchicine dose should be increased if patients have more than one FMF attack every 3 months and/or persistent elevation of inflammatory markers during the attack-free period. If a patient suffers from more than six typical FMF attacks per year, the patient should be accepted to be resistant to colchicine. The experts have suggested alternative treatment, such as IL-1 inhibitors, if there are persistent attacks despite the maximum doses of colchicine (2 mg in children; 3 mg in adults).

• #37 Cancer Incidence in Familial Mediterranean Fever – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/cancer-incidence-in-familial-mediterranean-fever/

  Familial Mediterranean Fever (FMF) is the most common hereditary monogenic fever syndrome characterized by recurrent attacks of fever and polyserositis. Anti-inflammatory drugs, with colchicine being the first-line therapy, have been used in FMF treatment to provide improvement in attacks and prevent amyloidosis, the most serious complication of the disease. […] Our findings suggest that Turkish FMF patients have a significantly lower incidence of cancer than the general population of Turkey. Patient characteristics, such as continuous exposure to anti-inflammatory drugs (primarily colchicine) and pathophysiological mechanisms of the disease can be interpreted for the explanation of this association.

• #38 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.

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