Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations

Lachmann, HJ; Şengül, Bülent; Yavuzşen, Tuğba; Booth, David R.; Booth, SE; Bybee, A; Gallimore, J. Ruth; Soytürk, Müjde; Akar, Servet; Tunca, Mehmet; Hawkins, Philip N.

doi:10.1093/rheumatology/kei279

Cited by 331 publications

(242 citation statements)

References 36 publications

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“…FMF is a disease in which subclinical inflammation can be seen, except during attack times (1,2,19). In our study, we found higher PWV values and frequency of AS in patients with FMF.…”

Section: Discussionsupporting

confidence: 59%

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Evaluation of arterial stiffness with plasma GGT levels and pulse wave velocity measurement in patients with FMF

et al. 2014

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Objective: Pulse wave velocity (PWV) is a non-invasive technique used to evaluate the arterial elasticity, which is an early indicator of atherosclerosis.Lately, gamma glutamyl transferase (GGT) is considered a determiner of arterial stiffness (AS). In this study, we aimed to evaluate the relationship between GGT levels and AS with PWV in patients with Familial Mediterranean fever (FMF). Material and Methods:The study was conducted with 60 patients with FMF and 40 controls. Genetic analysis of the patients were performed.AS was assessed by PWV and, after the measurement of PWV, the presence of AS was determined. Results:Mean PWV values and AS frequency were significantly higher in patients with FMF compared with the control group (p<0.001 and p=0.004, respectively). Mean GGT levels of FMF patients were higher than in the control group but the difference was not statistically different. In the correlation analysis, PWV and AS were positively correlated with FMF (r=0349, p<0.001; r=0.435, p<0.001, respectively). FMF duration and FMF were associated with GGT (r=0.300, p=0.02; r=0199, p=0.047, respectively). Conclusion:Increased PWV values in FMF patients may indicate arterial stiffness. These patients may be followed closely with PWV as an early indicator of atherosclerosis. Therefore, the cardiovascular risk can be determined in the early stages of disease and it may be possible to take necessary precautions.

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“…FMF is a disease in which subclinical inflammation can be seen, except during attack times (1,2,19). In our study, we found higher PWV values and frequency of AS in patients with FMF.…”

Section: Discussionsupporting

confidence: 59%

“…Recent studies have shown that subclinical inflammation may continue in some FMF cases, even in the symptom-free periods (1,2). Inflammation is an important cause of the development and progression of atherosclerosis (3).…”

Section: Introductionmentioning

confidence: 99%

Evaluation of arterial stiffness with plasma GGT levels and pulse wave velocity measurement in patients with FMF

et al. 2014

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“…Systemic AA amyloid deposition is always accompanied by increased SAA production, whether triggered by tissue damage, infection, or abnormal proinflammatory cytokine activity (26)(27)(28)(29)(30). The transgenic mice reported here allowed us to induce expression of the amyloidogenic mouse SAA isoform, SAA2, by oral administration of doxycycline, completely independently of any inflammatory stimulus.…”

Section: Discussionmentioning

confidence: 96%

Pathogenetic mechanisms of amyloid A amyloidosis

Simons

Al‐Shawi

Ellmerich

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

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Systemic amyloid A (AA) amyloidosis is a serious complication of chronic inflammation. Serum AA protein (SAA), an acute phase plasma protein, is deposited extracellularly as insoluble amyloid fibrils that damage tissue structure and function. Clinical AA amyloidosis is typically preceded by many years of active inflammation before presenting, most commonly with renal involvement. Using dose-dependent, doxycycline-inducible transgenic expression of SAA in mice, we show that AA amyloid deposition can occur independently of inflammation and that the time before amyloid deposition is determined by the circulating SAA concentration. High level SAA expression induced amyloidosis in all mice after a short, slightly variable delay. SAA was rapidly incorporated into amyloid, acutely reducing circulating SAA concentrations by up to 90%. Prolonged modest SAA overexpression occasionally produced amyloidosis after long delays and primed most mice for explosive amyloidosis when SAA production subsequently increased. Endogenous priming and bulk amyloid deposition are thus separable events, each sensitive to plasma SAA concentration. Amyloid deposits slowly regressed with restoration of normal SAA production after doxycycline withdrawal. Reinduction of SAA overproduction revealed that, following amyloid regression, all mice were primed, especially for rapid glomerular amyloid deposition leading to renal failure, closely resembling the rapid onset of renal failure in clinical AA amyloidosis following acute exacerbation of inflammation. Clinical AA amyloidosis rarely involves the heart, but amyloidotic SAA transgenic mice consistently had minor cardiac amyloid deposits, enabling us to extend to the heart the demonstrable efficacy of our unique antibody therapy for elimination of visceral amyloid.

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“…In a large proportion of patients symptom-free intervals of the disease are characterized by subclinical inflammation with overproduction of C-reactive protein (CRP), serum amyloid A (SAA) and other acute-phase reactants [28,29]. During attack-free periods, patients with FMF may still experience arthritis, myalgia, autonomic dysregulation with altered cardiovascular reactivity [30] and other cardiovascular symptoms related to persistent subclinical inflammation [18].…”

Section: Fmf As a Low-grade Inflammatory Disordermentioning

confidence: 99%

Familial Mediterranean Fever as an Emerging Clinical Model of Atherogenesis Associated with Low-Grade Inflammation

Yüksel¹,

Ayvazyan²,

Gasparyan³

2010

TOCMJ

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Numerous inflammatory and innate immune pathways are involved in atherogenesis. Elaboration of clinical models of inflammation-induced atherogenesis may further advance our knowledge of multiple inflammatory pathways implicated in atherogenesis and provide a useful tool for cardiovascular prevention. Familial Mediterranean fever (FMF) is a chronic inflammatory disorder with profiles of inflammatory markers close to that seen in the general population. In a few recent studies, it has been shown that endothelial dysfunction, increased atherosclerotic burden and activation of platelets accompany attack-free periods of FMF. Colchicine is proved to be useful in suppression of inflammation in FMF. Preliminary basic and clinical studies suggest that this relatively safe drug may be useful for cardiovascular protection in patients with FMF and in the general population. Multinational prospective studies are warranted to further elaborate clinical model of inflammation-induced atherosclerosis associated with FMF.

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Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations

Cited by 331 publications

References 36 publications

Evaluation of arterial stiffness with plasma GGT levels and pulse wave velocity measurement in patients with FMF

Evaluation of arterial stiffness with plasma GGT levels and pulse wave velocity measurement in patients with FMF

Pathogenetic mechanisms of amyloid A amyloidosis

Familial Mediterranean Fever as an Emerging Clinical Model of Atherogenesis Associated with Low-Grade Inflammation

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