Predictors of persistent inflammation in familial Mediterranean fever and association with damage

Babaoğlu, Hakan; Armağan, Berkan; Bodakçı, Erdal; Satış, Hasan; Ataş, Nuh; Sarı, Alper; Bilge, Nazife Şule Yaşar; Salman, Reyhan Bilici; Yardimci, G. K.; Güler, Aslıhan Avanoğlu; Karadeniz, Hazan; Kılıç, Levent; Öztürk, Mehmet Akif; Göker, Berna; Haznedaroğlu, Şeminur; Kalyoncu, Umut; Kaşifoğlu, Timuçin; Tufan, Abdurrahman

doi:10.1093/rheumatology/keaa378

Cited by 26 publications

(16 citation statements)

References 32 publications

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“…However, subclinical chronic inflammation continues during the attack-free period [21][22][23][24]. In our study, comparison of attack-free patients with the healthy control group revealed that CRP, S100A12, and ESR levels were significantly higher in attack-free patients than in the control group.…”

Section: Discussionmentioning

confidence: 42%

Evaluation of S100A12 protein levels in children with familial Mediterranean fever

Türkmenoğlu¹,

Güney²,

Bezen³

et al. 2021

Turk J Med Sci

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Background/aim: Familial Mediterranean fever (FMF), the most common autoinflammatory disease in children, is characterized by recurrent febrile episodes. FMF is known to progress with chronic inflammation, particularly during attack periods. This study aimed to investigate the relationship of S100A12, an inflammatory marker, with attacks and inflammatory events in FMF patients. Materials and methods: The study included 57 patients diagnosed with FMF, 43 in an attack-free period and 14 in an attack period, and 31 healthy children as the control group. Only white blood cell (WBC) count, C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR), and S100A12 level were analyzed in the control group. In addition, serum amyloid A (SAA), and fibrinogen levels were measured, and a mutation analysis was performed in the patient group. The results were compared among the attackfree period, acute attack FMF and control groups. Results: The mean age of patients and control group was 10 (2.5-18) and 9.5 (2.5-16) years, respectively. The CRP (p = 0.001), S100A12 (p = 0.003) and ESR (p= 0.001) values differed significantly between the FMF and control groups. S100A12 level (p = 0.027), WBC count (p = 0.003), CRP level (p = 0.0001), ESR (p = 0.004), and fibrinogen level (p = 0.001) differed significantly between the acute attack and attack-free period groups. SAA level (p = 0.05), ESR (p = 0.001), fibrinogen level (p = 0.001), WBC count (p = 0.001), and S100A12 level (p = 0.027) were higher in M694V homozygous FMF patients than in other FMF patients. 2 Conclusion: Patients with FMF had higher S100A12 levels than the control group, while the mean S100A12 concentration was higher in acute attack period patients than in attackfree period patients. S100A12 level might be an important indicator in the monitoring of chronic inflammation in patients with FMF.

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Section: Discussionmentioning

confidence: 42%

Evaluation of S100A12 protein levels in children with familial Mediterranean fever

Türkmenoğlu¹,

Güney²,

Bezen³

et al. 2021

Turk J Med Sci

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“…In a recent study by Babaoglu et al conducted with 917 FMF patients, the age of onset of the patients whose in ammation continued even during the attack free period was shown to present earlier; they were exposed to more frequent attacks of arthritis. It was found that this condition increased the development of amyloidosis by 3.59 fold, proteinuria development 3.28 fold, and kidney failure 4.18 fold (19). In addition, in early disease onset FMF patients, the international Severity of Scoring System (ISSF) was observed to be higher in FMF (25).…”

Section: Discussionmentioning

confidence: 96%

“…The presence of subclinical in ammation triggers many complications such as growth failure, puberty delay, and osteoporosis (19).…”

Section: Discussionmentioning

confidence: 99%

Early Disease Onset and Arthritis Are Predictors of Chronic Kidney Disease Development in FMF Patients

Büberci

Duranay

2021

Preprint

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Background: Familial Mediterranean fever (FMF) is an autosomal recessive genetic disease characterized by fever and serositis attacks. The most important complication is amyloidosis. In FMF patients, chronic kidney disease (CKD) can develop without amyloid development. The aim of the study is to evaluate the development of CKD in FMF patients and to determine the factors that are involved in this development.Method: One hundred seventy eight FMF patients who were followed up between 2000 and 2020 were included in the study. FMF diagnosis was made according to the Tel-Hashomer criteria. Genetic tests were studied in cases which there was suspicion of diagnosis. Clinical and demographic characteristics of patients and all laboratory data including urea, creatinine, estimated glomerular filtration rate (eGFR), and proteinuria in 24-hour urine at the time of first and last admission were evaluated.Results: The mean age of the patients was 34.53 ± 10.72, the follow-up period was 6.12 ± 3.94, and the diagnosis age was 21.7 ± 11.5 years. The number of patients with late disease onset and the percentage of kidney biopsy performed were higher in the genetic test group. There was no difference in the inflammatory parameters. The risk factors associated with the development of CKD were early disease onset and arthritis attacks.Conclusion: The role of genotype characteristics in the development of CKD has not been determined. Patients diagnosed with FMF disease at an early age and especially with arthritis attacks should be closely monitored in terms of the risk of developing CKD.

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“…The extent of renal damage defines the patients' prognosis, and renal biopsy is the most common diagnostic test revealing amyloid deposits. The majority of reports on FMF-related AA amyloidosis are retrospective in nature or case series [2][3][4][5][6][7]. The pathogenesis and risk factors for amyloidosis in FMF remain partially understood.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of E148Q and Concomitant AA Amyloidosis in Patients with Familial Mediterranean Fever

Arıcı

Romano

Piskin

et al. 2021

JCM

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The aim of the study was to compare the clinical phenotype of patients with familial Mediterranean fever (FMF)-related AA amyloidosis, according to the age of FMF diagnosis and E148Q genotype. Patients with biopsy-confirmed FMF-related AA amyloidosis were included in the study. Tel-Hashomer criteria were applied in the diagnosis of FMF. All patients had detailed baseline assessment of clinical features, renal functions, genetic testing, histopathological diagnosis of amyloidosis, and treatment received. Multiple comparisons were performed according to the age of diagnosis, disease phenotype, mutation, and mortality. Our study included 169 patients with a diagnosis of AA amyloidosis. There were 101 patients diagnosed with FMF < 18 years of age and 68 patients diagnosed who were ≥18 years of age. The three most common clinical manifestations were fever (84.6%), abdominal pain (71.6%), and arthritis (66.9%). The most common allele among FMF patients was M694V (60.6%), followed by E148Q (21.4%), and M680I (10.3%). The most frequent genotypes were M694V/M694V (45.0%), M694V/E148Q (14.8%), and E148Q/E148Q (11.2%) among 169 patients in our cohort. During the follow-up period, 15 patients (10 male, 5 female) died, of whom 14 had M694V homozygous genotype and one was homozygous for E148Q. Clinicians should be aware of patients with homozygous E148Q genotype for close monitoring and further evaluation. The possible relationship between E148Q and AA amyloidosis needs to be confirmed in other ethnicities.

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Predictors of persistent inflammation in familial Mediterranean fever and association with damage

Cited by 26 publications

References 32 publications

Evaluation of S100A12 protein levels in children with familial Mediterranean fever

Evaluation of S100A12 protein levels in children with familial Mediterranean fever

Early Disease Onset and Arthritis Are Predictors of Chronic Kidney Disease Development in FMF Patients

Evaluation of E148Q and Concomitant AA Amyloidosis in Patients with Familial Mediterranean Fever

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