Chronic inflammation in FMF: markers, risk factors, outcomes and therapy

Ben-Zvi, Ilan; Livneh, Avi

doi:10.1038/nrrheum.2010.181

Cited by 198 publications

(162 citation statements)

References 68 publications

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“…This condition is known as Uhthoff's phenom-enon [32]. It is explained by a conduction block because of the ionic channels properties change under high temperature [33]. However, the effect of heat changes on MS can be multi-factorial.…”

Section: Discussionmentioning

confidence: 99%

The Potential Negative Effects of Interleukin-1β in Multiple Sclerosis Patients with MEFV Mutation

Alpaycı¹

2014

Ann Clin Anal Med

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ÖzetMEditerranean FeVer (MEFV) gen mutasyonlu multipl skleroz hastaları, bu mutasyonu taşımayanlara kıyasla daha hızlı ilerleyen hastalığa sahiptirler.Ancak, bunun mekanizması henüz tam olarak aydınlatılamamıştır. Bu yazı, bu hızlı ilerlemeden sorumlu olabilen interlökin-1β (IL-1β)'nın muhtemel rolünü önermektedir. MEFV geni, pyrin proteinini kodlar ve mutasyonları ailesel Akdeniz ateşine neden olur. MEFV mutasyonları, pyrin fonksiyonunun ortaya çıkmasına yol açar ve IL-1β'nın uygun olmayan salınımıyla sonuçlanır. gene mutation, have faster progression than the non-carriers. However, its underlying mechanism is not well understood. This article proposes the potential role of interleukin-1β (IL-1β) that may be responsible for this rapid progression. Mutations in MEFV, the gene encoding for protein pyrin, cause familial Mediterranean fever, lead to gain of pyrin function, resulting in inappropriate IL-1β release. Interleukin-1β is a major mediator of systemic inflammation and fever, and also it contributes to permeability of the bloodbrain barrier in active lesions of multiple sclerosis. Moreover, IL-1β promotes apoptosis of neurons and oligodendrocytes that produce the myelin sheath, which insulates axons. Thus, inflammatory damage, the blood-brain barrier disfunction, effects of fever on the central nervous system (or Uhthoff's phenomenon), and apoptosis of neurons and oligodendrocytes, which play an important role in the pathogenesis and clinical course of multiple sclerosis, can be induced by increased activation and release of IL-1β in the presence of MEFV gene mutations. Therefore, screening for MEFV mutations in patients with multiple sclerosis and treatment planning with IL-1β targeting drugs for the carriers, may be a logical idea that will be a source of inspiration for scientific studies.

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Section: Discussionmentioning

confidence: 99%

The Potential Negative Effects of Interleukin-1β in Multiple Sclerosis Patients with MEFV Mutation

Alpaycı¹

2014

Ann Clin Anal Med

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“…AA amyloidosis is the most devastating complication of FMF (7). Despite colchicine prophylaxis, amyloidosis may still prevail in about 5% of FMF patients, leading eventually to end-stage kidney disease in almost all patients.…”

Section: Introductionmentioning

confidence: 99%

“…Clinically, FMF is characterized by self-limited attacks of severe inflammation with fever and sterile serositis, each lasting up to 4 days (3)(4)(5)(6). Chronic inflammation and its sequelae, including anemia, splenomegaly, continuously elevated acute-phase reactants, and amyloidosis may also occur in 30-60% of untreated or treatment-refractory patients (7). The MEditerranean FeVer gene (MEFV) was cloned in 1997, but only recently was it found that the protein encoded by it (pyrin/marenostrin) is a sensor protein of bacterial antigens that is used to promote inflammation (8,9).…”

Section: Introductionmentioning

confidence: 99%

Late ventricular potentials in familial Mediterranean fever with and without AA amyloidosis

Nussinovitch¹,

Livneh²

2017

Eur J Rheumatol

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Data on increased rhythm and conduction disturbances in FMF are limited and conflicting. For instance, Akcay et al. (13) found that QT dispersion (QTd) and corrected dispersion (QTcd), which reflect cardiac repolarization heterogeneity and thereby suggest predisposition for cardiac arrhythmias, are increased in uncomplicated FMF. However, different methodologies have yielded normal QTd results in both uncomplicated and amyloidosis-affected FMF patients (14, 15). Limited information is found on depolarization-associated markers for arrhythmias. Low-amplitude, high-frequency waves-termed late ventricular potentials (LPs)-can be detected immediately adjacent to the QRS complex by signal-averaged electrocardiography Udi Nussinovitch 1 , Avi Livneh 2,3Original Article Abstract Objective: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by episodic and chronic inflammation that may lead to both accelerated coronary atherosclerosis and cardiac AA amyloidosis. We hypothesized that late ventricular potentials (LPs), an established electrocardiographic susceptibility marker of ventricular arrhythmias, will be more common in FMF than in the adjusted normal population due to these two types of inflammation-associated cardiac effects. Therefore, we aimed to evaluate the occurrence of LPs in FMF patients with and without amyloidosis.Material and Methods: Signal-averaged electrocardiography was performed in consecutive patients with FMF using the Frank corrected orthogonal lead system. At least 200 consecutive beats were digitally recorded and averaged, and the presence of LPs was determined according to acceptable thresholds.Results: There were 54 patients with colchicine-treated FMF, of whom 14 had biopsy-proven AA amyloidosis. None of the uncomplicated FMF patients and 2 of the 14 FMF amyloidosis patients had abnormal or borderline LPs. Conclusion:Based on LPs as a susceptibility marker for arrhythmia, FMF patients, including the large majority of FMF patients with amyloidosis, are seemingly not at an increased risk to develop arrhythmias.

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“…In FMF patients, the effusion of neutrophils in serous cavities causes infl ammatory attacks, which results in increase in acute phase reactants [1]. Various studies have reported that the levels of interleukin-6 (IL-6), interleukin-10 (IL-10), and tumor necrosis factor-alpha (TNF-) increase during and between FMF attacks [7,8].…”

Section: Introductionmentioning

confidence: 99%

“…Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by recurrent fever, abdominal pain, monoarthricular and/or oligoarthricular arthritis, polyserositis, and erysipelas-like erythema [1] .FMF has been predominantly found in ethnic groups living around the Mediterranean basin (Jews, Arabs, Turks, and Armenians) [1].…”

Section: Introductionmentioning

confidence: 99%

The Coexistence of Familial Mediterranean fever and Fibromyalgia Syndrome: Two Cases Reports

Tural¹

2017

Open J Orthop Rheumatol

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Familial Mediterranean Fever (FMF) is an autoinfl ammatory disease caused by mutations in the MEFV gene and characterized by recurrent fever, polyserositis and arthritis. It is transmitted in an autosomal recessive pattern. FMF has been predominantly found in ethnic groups living around the Mediterranean basin (Jews, Arabs, Turks, and Armenians). Although accompanying infl ammatory diseases have been reported in FMF, the coexistance of fi bromyalgia syndrome (FMS) is very rare. MEFV gene analysis that the fi rst patient had compound heterozygous mutation (M680I+V726A) and the other patient had heterozygous mutation (M694V). This association will be discussed in this case report.

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Chronic inflammation in FMF: markers, risk factors, outcomes and therapy

Cited by 198 publications

References 68 publications

The Potential Negative Effects of Interleukin-1β in Multiple Sclerosis Patients with MEFV Mutation

The Potential Negative Effects of Interleukin-1β in Multiple Sclerosis Patients with MEFV Mutation

Late ventricular potentials in familial Mediterranean fever with and without AA amyloidosis

The Coexistence of Familial Mediterranean fever and Fibromyalgia Syndrome: Two Cases Reports

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