Fibrinogen-to-Albumin Ratio in Familial Mediterranean Fever: Association with Subclinical Inflammation

2021 ◽  
Author(s):  
Esra Nagehan Akyol Onder ◽  
Pelin Ertan

Abstract Background Familial Mediterranean fever (FMF) is the most seen monogenic periodic fever syndrome characterised by bouts of fever and serositis. It is known that subclinical inflammation (SI) can persist in the symptom-free period and lead to amyloidosis even under colchicine treatment. This study aimed to evaluate the role of the fibrinogen-to-albumin ratio (FAR) in FMF and its correlation with SI. Material and Methods A total of 112 patients with FMF and 78 controls were enrolled in this retrospective study. Demographic, laboratory and genetic data were obtained from the hospital records. Results The FAR values of the FMF cases were significantly higher than the control group (p<0.001). In the FMF group, the patients with SI had higher FAR values than those without SI (p<0.001). FAR was positively correlated with SI (r=0.413, p<0.001). The receiver operating characteristic curve analysis showed that FAR had a higher area under the curve value than albumin and fibrinogen. Conclusion Detecting SI in patients with FMF is crucial in preventing amyloidosis, the most devastating complication of FMF. FAR is a simple, inexpensive, easily obtained indicator which can be used for reflecting SI in FMF.

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Tomonobu Sato ◽  
Shunichiro Takezaki ◽  
Takeru Goto ◽  
Shinichi Ishikawa ◽  
Kazumi Oura ◽  
...  

Periodic fever syndromes are heterogeneous diseases. Familial Mediterranean fever (FMF) is one of the hereditary periodic fever diseases caused by a Mediterranean fever (MEFV) gene abnormality. FMF can be categorized as typical or atypical, based on clinical findings and genetic screening. Atypical FMF has a wide variation of clinical findings and disease-causing mutations of MEFV. Therefore, it is sometimes difficult to diagnose an unknown fever as FMF. To date, a large number of various typical and atypical FMF cases have been reported in Japan. Here, we describe a Japanese boy with heterozygous MEFV p.Ser503Cys exon 5 variant who developed periodic fever. He was treated with colchicine; a complete eradication of his fever and various accompanying symptoms have been subsequently achieved for more than a year. Given that there have been a few reports about patients with this variant, little is known about the genetic and phenotypic role of heterozygous MEFV p.Ser503Cys exon 5 variant. It is therefore imperative to consider atypical FMF as a differential diagnosis when a periodic fever is encountered. Furthermore, we suggest that it is worthwhile to integrate MEFV gene analysis with the potential effects of colchicine treatment in patients with periodic fever.


2021 ◽  
Author(s):  
Esra Nagehan Akyol Onder ◽  
Fethi Sirri Cam ◽  
Pelin Ertan

Abstract Background Familial Mediterranean Fever (FMF), which is characterised by recurrent episodes of fever with serositis, is associated with ongoing inflammation without clinical findings during attack-free periods, leading to amyloidosis, the most important complication of FMF. The objective of this study was to investigate the C-reactive protein/albumin ratio (CAR) as a marker to identify subclinical inflammation in symptom-free FMF children and compare the CAR with other systemic inflammatory markers such as mean platelet volume (MPV), red cell distribution width (RDW), neutrophil/lymphocyte ratio (NLR) and platelet/lymphocyte ratio (PLR). Material and Methods We included 100 patients and 70 healthy subjects. Hospital records were obtained to collect data on laboratory findings and genetic mutations. Results We found that the CAR levels of our FMF patients were significantly higher than those of the control group. We also evaluated that the CAR values had a higher area-under-the-curve value than the other systemic inflammation parameters including CRP, MPV, RDW, NLR, PLR based on Receiver-Operating Characteristics (ROC) analysis. Conclusion It is important to identify subclinical inflammation in FMF patients with simple, reliable, easily accessible markers to avoid amyloidosis. Although the CAR might be used to assess subclinical inflammation in paediatric FMF patients, the prognostic value of CAR is not superior to CRP. Merging CRP and albumin into a single index thus provides no additional benefit in detecting subclinical inflammation in FMF.


2021 ◽  
Vol 16 (1) ◽  
pp. 1365-1376
Author(s):  
Yiping Cheng ◽  
Wenhao Yu ◽  
Yuping Zhou ◽  
Tao Zhang ◽  
Haiyan Chi ◽  
...  

Abstract The role of inflammation has been identified in the pathogenesis of diabetic ketoacidosis (DKA). The neutrophil/lymphocyte ratio (NLR) and white blood cells (WBC) can be used to predict a systemic inflammatory response. Changes in NLR and WBC levels have never been explored in type 1 diabetes mellitus (T1DM) patients with DKA and an uninfected state. This retrospective study included a total of 644 participants. NLR and WBC were measured in the control group (n = 316) and in T1DM patients with mild-DKA (n = 92), severe-DKA (n = 52), and non-DKA (n = 184) in an uninfected state. Then, we assessed the independent predictors of DKA occurrence in T1DM patients in an uninfected state. The diagnostic performance of variables was determined by receiver operating characteristic curve analysis. Serum NLR of T1DM patients is significantly higher than that of normal controls, and if DKA occurs, NLR increases further and increases with the severity of DKA. In addition to diastolic blood pressure, blood urea nitrogen, glycated hemoglobin (HbA1c), and WBC, NLR was also independently associated with DKA in T1DM patients with an uninfected state (OR = 1.386, 95% CI: 1.127–1.705, p = 0.002). Furthermore, the diagnosis analysis showed that except for NLR and WBC, the area under the curve (AUC) of indicators with a statistical difference in patients with and without DKA were 0.747 for DKA diagnosis, and after the addition of NLR and WBC, the AUC was 0.806. The increased NLR level represents a low-cost and highly accessible predictor for DKA in T1DM patients with an uninfected state. The addition of inflammation indicators can play a statistically significant role in the prediction model of the DKA occurrence.


2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
E. Verrecchia ◽  
L. L. Sicignano ◽  
M. La Regina ◽  
G. Nucera ◽  
I. Patisso ◽  
...  

Objective. Familial Mediterranean fever (FMF) is an autosomal recessive disease due to a MEFV gene mutation. Since Helicobacter pylori infection has been described to increase the severity and frequency of FMF attacks, we evaluate if overgrowth of small intestinal bacterial (SIBO), associated with a release of bacterial products, can affect the response to colchicine in FMF patients poorly responsive to colchicine. Methods. We revised our Periodic Fever Centre database to detect FMF patients who were poorly responsive to colchicine, without a well-defined cause of drug resistance. They were evaluated for SIBO presence, then treated with decontamination therapy. Results. Among 223 FMF patients, 49 subjects show colchicine resistance, and no other known causes of colchicine unresponsiveness has been found in 25 patients. All 25 patients underwent glucose breath test; 20 (80%) of them were positive, thus affected by SIBO. After a successful decontamination treatment, 11 patients (55%) did not show FMF attacks during the following three months (p<0.01), while 9 of them revealed a significant reduction of the number of attacks compared to three months before (p<0.01). Conclusion. The SIBO eradication improves laboratory and clinical features of FMF patients. Thus, patients with unresponsiveness to colchicine treatment should be investigated for SIBO.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Desheng Yang ◽  
Haini Li ◽  
Xiaoyan Sun ◽  
Shengmei Yang ◽  
Kewei Wang ◽  
...  

AbstractThe purpose of the present study was to evaluate the diagnostic role of CRP in ovarian cancer and to assess whether CRP can be combined with tumor markers to enhance the diagnostic efficacy toward ovarian cancer. Area under the curve, sensitivity, and specificity were calculated to access the diagnostic ability of each singly and combined as markers for ovarian cancer. The CRP cut-off value was then calculated to evaluate the diagnostic efficacy of CRP for ovarian cancer. Our results showed that values for all markers were significantly higher in the cancer group than in the control group. Receiver operating characteristic curve results showed that CA125 had the highest diagnostic efficacy for ovarian cancer, while the sensitivity for CRP was higher than for CA125, and the specificity for CRP was equal to that of CA125. The combination of CRP, CA125, and HE4, however, provided the strongest diagnostic capability. Furthermore, the diagnostic cut-off value for CRP with regard to ovarian cancer was 9.8 mg/L, and high levels of CRP were correlated with stage and tumor size of ovarian cancer. Our study indicated that CRP is valuable in the diagnosis of ovarian cancer, and that combining CRP with CA125 and HE4 improved the diagnostic efficacy with respect to ovarian cancer.


2020 ◽  
pp. jrheum.200574
Author(s):  
Pavel Olegovich Sotskiy ◽  
Olga Leontevna Sotskaya ◽  
Hasmik Sureni Hayrapetyan ◽  
Tamara Fadei Sarkisian ◽  
Anna Rafaelovna Yeghiazaryan ◽  
...  

Objective Recurrent attacks of peritonitis of Familial Mediterranean fever (FMF), may lead to peritoneal adhesions and fallopian tube obstruction. Colchicine - the treatment of choice for FMF - may disturb cell division. Secondary amyloidosis - a complication of untreated FMF - may involve the testes and ovaries. Thus, FMF and colchicine may potentially affect fertility and pregnancy in FMF patients. The aims of the study are to evaluate the causes of infertility and pregnancy outcome in FMF patients and to compare them with two groups: non-FMF patients with peritoneal female genital tuberculosis (FGTB) and normal healthy control. Methods This is a retrospective study in which FMF patients with reproductive disorders were recruited from the National Center of Medical Genetics and Primary Health Care in Yerevan, Armenia. The FGTB patients and the normal control patients with reproductive problems were recruited successively from a large gynecology clinic in Yerevan. Genetic analyses for FMF were performed using ViennaLab Diagnostics GmbH Strip Assay. Results The FMF group (211 patients) resembles the FGTB group (127 patients) regarding etiologies of infertility. However, in vitro fertilization (IVF) success rate and pregnancy outcome were comparable between the FMF patients and the control group (167patients). Infertility in FMF patients was clearly associated with a more severe disease and a lack of adequate colchicine treatment. Conclusion Colchicine medication and controlled FMF disease do not adversely affect the reproductive system and pregnancy outcome. However, a lack of an appropriate colchicine treatment may cause infertility and poor pregnancy outcome.


2020 ◽  
Vol 58 (1) ◽  
pp. 27-33
Author(s):  
Meliha Bayram ◽  
Mehmet Emin Derin ◽  
Halef Okan Doğan ◽  
Gökmen Asan ◽  
Mehtap Şahin ◽  
...  

AbstractIntroduction. Familial Mediterranean Fever (FMF) is an autoinflammatory disease. Prolidase is a specific imidodipeptidase that plays a role in collagen degradation, and an important role in inflammation and wound healing. Hypoxia-inducible factor-1α (HIF-1) is an important protein in the regulation of immunological response, hemostasis, vascularization. The aim of the study was to compare serum prolidase and HIF-1α levels in patients with FMF in attack-free period and healthy control group.Methods. Between August 2017 and December 2017, sixty patients diagnosed with FMF according to the criteria of the Tel-hashomer and admitted to Sivas Cumhuriyet University Medical Faculty, Internal Medicine Rheumatology Department and sixty healthy volunteers were enrolled in the study.Results. Median serum prolidase levels were 72.1 (25.1–114.9) ng/ml in FMF group and 30.7 (21.3–86.2) ng/mL in healthy control (HC) group (p = 0.018). ROC analysis showed that the sensitivity was 65% and the specificity was 68.3% at serum prolidase levels 54.03 ng/mL (p < 0.05). The median serum levels of HIF-1α in the FMF group was 482.0 (292.0–3967.0) pg/mL and 632.0 (362.0–927.0) pg/mL in the HC group (p > 0.05). There was no significant correlation between laboratory findings, sex, age, and prolidase (p > 0.05).Conclusion. Serum prolidase enzyme levels in FMF patients with attack-free period were significantly higher than in the HC group. However, the role of prolidase and HIF1-α in the FMF disease needs to be clarified with more extensive and comprehensive studies.


2021 ◽  
Vol 14 (3) ◽  
pp. 1351-1358
Author(s):  
Rania Fawzy Mahmoud Abdelkawy ◽  
Shams Kholoussi ◽  
Eman Eissa ◽  
Khaled Hamed ◽  
Haiam Abdel Raouf ◽  
...  

Background: Familial Mediterranean fever (FMF) is an auto inflammatory genetic disease resulted from the mutation of pyrin, which contributes to the formation of inflamma some complex. Therefore, activation of cytokines is one of the hallmarks of FMF pathogenesis. This study aimed to investigate the role of miRNAs as regulatory biomarkers for inflammation in patients with FMF. Methods: 50 FMF patients and 25 healthy subjects were included in this study. Q RT-PCR was used to determine plasma expressions of miR-181a and miR-125a, while IFN-γ and IL-17 were estimated using ELISA technique. Results: Our results indicated that, the expression of miR-181a was significantly decreased (p = 0.006) while miR-125a expression was insignificantly reduced (p = 0.101) also IL-17 levels were significantly higher(p = 0.003) and plasma IFN-γ levels were insignificantly increased (p = 0.322) in FMF patients than control group. Correlation analysis revealed a positive correlation between miR-181a expression and lymphocyte percentages (p = 0.048),while a significant negative association was observed between miR-125a and C-reactive protein (CRP) (p = 0.005) in FMF patients. However, there were no associations between miR-125a and miR-181a with IFN-γ and IL-17 in FMF patients. Conclusion: miR-181a and miR-125a could be used as regulatory biomarkers for inflammation in FMF patients.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1808.2-1809
Author(s):  
D. Karatas ◽  
Z. Öztürk ◽  
D. Cekic ◽  
Z. Yuertsever ◽  
Ü. Erkorkmaz ◽  
...  

Background:Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent attacks of fever, peritonitis, pleuritis, arthritis, and skin eruption (1). It is shown by studies that chronic diseases like diabetes mellitus, chronic heart disease, hypertension which other than inflammatory – rheumatologic disease increase depression and anxiety (2). There are a few studies evaluating depression and anxiety in FMF patients, and these results are conflicting (3,4).Objectives:To assess the frequency of depression and anxiety in patients with Familial Mediterranean Fever (FMF)Methods:In this study, 77 FMF patients aged 18 and over who were followed up in Sakarya University Education and Research Hospital, Department of Rheumatology, and 78 healthy volunteers aged 18 and over as thecontrol group. Beck depression scale and Beck anxiety scale were used to depression and anxiety, respectively. Beck’sdepression scale was evaluated as 9 and below normal, 10-16 mild depression, 17-29 moderate depression, 30-63 severe depression. Beck anxiety scale was evaluated as 0-8 normal, 8-15 mild anxiety, 16-25 moderate anxiety, 26 and above severe anxiety.FMF disease severity was determined by Pras scoring.Results:The study group, comprised 77 diagnosed with FMF with a meanage of 37.18 and a control group comprised of 78 healthy controls (C) with a meanage of 35.32 (p=0,058). İn studygroup (P) %63.6, control group (C) %53.8 as female. %36.4 of thestudy group(C), %46.2 of the control group are male. (p=0,216). The prevalence of depression was significantly higher in FMF patients compared to the control group (in order P;C: normal %24,7; %47,4, mild depression: %40.3; %26.9, moderate depression %26; %19.2, severe depression %11.7; %6.4 p<0.015). Similarly in depression results; the prevalence of anxiety was significantly higher in FMF patients compared to the control group (in order P;C normal %23,4; %57.7, mild anxiety %26; %20.5, moderate anxiety %26; %15.4, severe anxiety %24.4; %6.4 p<0,001). Depression status was not correlated with FMF disease severity (p=0.645). A correlation was found between FMF severity and anxiety which it is which was found statistically significant (p=0.005).There was no relationship between erythrocyte sedimentation rate and C-reactive protein with depression and anxiety.Conclusion:Both anxiety and depression frequency are increased in FMF patients compared to healthy controls.References:[1]Livneh A, Langevitz P, Zemer D et al. (1997) Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 40 (10), 1879–85.[2]Alonso J, Ferrer M, Gandek B, Ware JE Jr, Aaronson NK, Mosconi P, Rasmussen NK, Bullinger M, Fukuhara S, Kaasa S, Leplège A, IQOLA Project Group (2004) Health-related quality of life associated with chronic conditions in eight countries: results from the International Quality of Life Assessment (IQOLA) Project. Qual Life Res 13:283–298[3]Makay B, Emiroglu N, Unsal E (2010) Depression andanxiety in children and adolescents with familial Mediterranean fever. Clin Rheumatol 29, 375–9.[4]Giese A, Ornek A, Kilic L, Kurucay M, Sendur S. N., Lainka E, Henning B. F. Anxiety and depression in adult patients with familialMediterranean fever: a study comparing patients living in Germany and Turkey. International Journal of Rheumatic Diseases 2017; 20: 2093–2100Disclosure of Interests:None declared


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