The frequency of synovitis and calcium pyrophosphate deposition with ultrasound is increased in transfusion-dependent beta-thalassaemia patients: effect of iron accumulation?

OBJECTIVES: To determine the frequency of synovitis and calcium pyrophosphate deposition (CPDD) with ultrasound (US) in the wrists of transfusion dependant (TD) beta-thalassaemia patients and to investigate the associated factors with these pathologies. METHODS: Eighty-seven beta-thalassaemia patients (46 thalassaemia major and 41 thalassaemia minor patients) were grouped into two as TD and transfusion non-dependent (TND)-thalassaemia patients. Under bilateral wrist US the presence of synovial hypertrophy (SH), power Doppler signal (PD) combined synovitis (SH+PD), tenosynovitis, and triangular fibrocartilage complex (TFC)-cartilage calcification (CC) were examined. SH, PD, and combined synovitis in the US were classified as Grade-0 (no), Grade-1 (minimal), Grade-2 (moderate), and Grade-3 (severe). RESULTS: The incidence of moderate/severe SH, PD, and combined synovitis with US was 34.8%, 17.4%, and 34.8% in TD-thalassaemia patients, respectively, but none in TND patients (p<0.001, p=0.006, p<0.001). The frequency of TFC-CC with US was 32.6% in TD and 2.4% in TND-thalassaemia patients (p<0.001). Ferritin level was positively correlated with SH (r=0.414, p<0.001), PD (r=0.279, p=0.009) and combined synovitis scores (r=0.402, p<0.001). Ferritin level (OR:1.001, CI:1.000-1.002) and the presence of TFC-CC (OR:25.048, CI:5.187-120.951) were determined as to be associated with moderate/severe combined synovitis. CONCLUSIONS: The presence of synovitis and TFC-CC with the US is common in patients with beta-thalassaemia who have had recurrent blood transfusions. Iron overload in beta-thalassaemia patients may cause CPDD and synovial inflammation.

Evaluation of thiol/disulfide hemostasis and serum Ischemia modified albumin as oxidative stress biomarkers in systemic lupus erythematosus patients: Relationship with major organ involvement and disease activity.

OBJECTIVES: We aimed to compare thiol/disulfide hemostasis and serum ischemia-modified albumin (IMA) levels, which are indicators of oxidative stress (OS), in patients with systemic lupus erythematosus (SLE), with the healthy control (HC) group and to evaluate the relationship of these parameters with disease activity and major organ involvement. MATERIAL-METHODS: Eighty-four SLE patients and 96 HCs were included in this study. The disease activity of SLE patients was calculated using The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K). Patients with SLEDAI-2K ≤ 5 were classified as low disease activity (LDA) and those with SLEDAI-2K > 6 as high disease activity (HDA). Thiol/disulfide hemostasis was evaluated using a new automated method and natural thiol (NT), total thiol (TT), disulfide (SS) levels, SS/NT, SS/TT, NT/TT ratios, and serum IMA levels were recorded. RESULTS: NT and TT levels were significantly lower (490.11 ± 123.61 vs 536.96 ± 86.05, p = 0.003) (532.56 ± 125.80 vs 565.72 ± 89.82, p = 0.046), SS level (21.22 ± 11.75 vs 13.37 ± 9.31, p < 0.001) was higher, and SS/TT (4.64 ± 2.93 vs 2.52 ± 1.82, p < 0.001) and SS/NT (4.12 ± 2.33 vs 2.35 ± 1.59, p < 0.001) ratios were significantly higher in SLE patients compared to HCs. IMA values were not different between the two groups (p = 0.920). NT (449.84 ± 136.98 vs 520.32 ± 104.11, p = 0.012) and TT levels (492.01 ± 138.45±562.97 ± 107.09, p = 0.013) were significantly lower and serum IMA levels (0.802 ± 0.089 vs 0.764 ± 0.040, p = 0.023) were significantly higher in SLE patients with HDA than in LDA patients. There was a weak negative correlation between NT (r = -0.284, p=0.009) (r = -0.291, p = 0.007) and TT levels (r = -0.281, p = 0.010) (r = -0.289, p = 0.008) and a weak positive correlation between IMA levels (r = 0.279, p = 0.011) (r = 0.263, p = 0.016) and SLEDAI-2K, and major organ involvement. CONCLUSION: It is thought that thiol/disulfide hemostasis and IMA levels may be used as ideal biomarkers of OS in SLE patients and may reflect the disease activity and major organ involvement.

Central sensitisation in primary Sjögren Syndrome and its effect on sleep quality.

OBJECTIVE: The aim of the present study is to evaluate the presence and frequency of central sensitisation (CS) in primary Sjögren Syndrome (pSS) and to determine the effect of CS on sleep quality. MATERIALS AND METHODS: In this cross-sectional study, 50 patients diagnosed with pSS between the ages of 18 and 75 were included. The healthy control group was composed of 43 healthcare workers. Each participant underwent a physical examination, and demographic data and the medications they used were recorded. Central sensitisation inventory and Pittsburgh Sleep Quality Index questionnaires were filled in to garner data on CS and sleep quality, respectively, from all participants. RESULTS: While central sensitisation inventory >40 was detected in 74% of pSS patients, it was 25.6% in healthy controls, and there is a statistically significant difference between the groups (p < .05). A correlation analysis of the central sensitisation inventory and Pittsburgh Sleep Quality Index values of all participants revealed a statistically significant correlation between all parameters other than the duration of sleep (p < .05). CONCLUSIONS: CS was found to have a negative effect on sleep quality in patients with pSS. We suggest that the cause of widespread pain seen in patients with pSS as the possible development of CS should be considered.

Evaluation of retinal microvascular structures by optical coherence tomography angiography in primary Sjögren's syndrome.

OBJECTIVES: There are insufficient data in the literature on how retinal capillaries are affected in primary Sjögren's syndrome (PSS). The aim of this study was to evaluate the retinal capillary density (CD) in PSS using optical coherence tomography angiography (OCTA). METHODS: In this case-control study, 26 eyes from 13 PSS patients and 39 eyes from 20 healthy controls (HCs) were included. The CD in the regions of the superior capillary plexus (SCP), deep capillary plexus (DCP) and radial peripapillary capillaries (RPC) as well as assessment parameters of the foveal avascular zone (FAZ) were examined by OCTA. RESULTS: The mean CD (%) was 50.2 ± 4.2 and 50.5 ± 3.4 in the SCP (p = 0.904), 49.2 ± 7.5 and 53.9 ± 5.7 in the DCP (p = 0.006) and 50.8 ± 2.1 and 49.8 ± 2.2 in the RPC (p = 0.088) regions in patients with PSS and HCs, respectively. In patients with PSS and HCs, the mean sizes of the FAZ were 0.243 ± 0.07 mm2 and 0.283 ± 0.13 mm2 (p = 0.142), and the mean sizes of the non-flow area were 0.480 ± 0.11 mm2 and 0.509 ± 0.13 mm2, respectively (p = 0.359). The correlation coefficients (Rho) of retinal CD in the SCP, DCP and RPC regions with disease duration were - 0.545 (p = 0.004), - 0.389 (p = 0.050) and - 0.795 (p < 0.001), respectively. CONCLUSION: The retinal CD in PSS is lower than that in the healthy population in deep retinal capillaries, and retinal CD shows a negative correlation with disease duration in PSS. CLINICAL TRIALS REGISTRATION: This study was not registered to clinicaltrials.gov.

Central sensitization and its relationship with health profile in Behçet's disease.

OBJECTIVES: In this study, we aimed to evaluate the level of central sensitization (CS) and its relationship with health profile, including neuropathic pain and sleep quality in Behçet's disease (BD). METHOD: Eighty-eight patients with BD and 60 healthy controls (HCs) were included in the study between May 2018 and February 2019. Nottingham health profile (NHP), pain DETECT, Pittsburgh sleep quality index (PSQI) questionnaires and the central sensitization inventory (CSI) were administered to all participants. To evaluate the correlations of the NHP, PSQI, and PDQ scores with the CSI score, the CSI was modified for each questionnaire. The activity of BD was determined by the Behçet's disease current activity form (BDCAF). RESULTS: CSI scores were significantly higher in patients with BD than HCs (BD: 41.2 ± 21.1 vs HCs: 20.4 ± 16.9, p < .001). Clinical CS was detected in 69.3% of patients with BD and 28.3% of HCs (p < .001). Severe or extreme CS (CSI score ≥ 50) was present in 37.5% of patients with BD and 5.0% of HCs (p < .001). There were high correlations between the modified CSI scores and the NHP, PDQ and PSQI scores in patients with BD (R = 0.804; p < .001, Rho = 0.698; p < .001, and Rho = 0.734; p < .001, respectively). There was significant correlation between CSI and BDCAF scores (Rho= 0.470, p < .001). CONCLUSION: CS is more frequent and more severe in patients with BD than in HCs. There is a strong correlation between the severity of CS and poor health profile in patients with BD.

Evaluation of retinal vascularization by optical coherence tomography angiography (OCTA) in rheumatoid arthritis, and its relationship with disease activity.

PURPOSE: To measure the retinal capillary density quantitatively with optical coherence tomography angiography (OCTA) in patients with rheumatoid arthritis (RA) and healthy controls (HCs), and to evaluate the relationship between OCTA findings and RA disease activity. METHODS: In this cross-sectional study, 106 eyes of RA patients and 71 eyes of HCs were evaluated. RA patients were divided into inactive (DAS28 < 3.2) and active (DAS28 ≥ 3.2) subgroups. Retinal capillary plexus density (CPD) was obtained from the superficial capillary plexus (SCP), deep capillary plexus (DCP), and radial peripapillary capillary (RPC). RESULTS: In RA patients and HCs, the CPD (%) was 50.99 ± 3.30 and 52.08 ± 2.36 (p = .013) in the SCP, 55.65 ± 5.73 and 57.53 ± 4.60 (p = .019) in the DCP, and 49.98 ± 2.25 and 49.93 ± 2.25 (p = .947) in the RPC blood supply regions, respectively. In inactive and active RA patients, the CPD (%) was 51.01 ± 2.92 and 50.97 ± 3.73 (p = .947) in the SCP, 55.02 ± 5.70 and 56.40 ± 5.74 in the DCP (p = .229), and 50.34 ± 2.23 and 49.55 ± 2.22 (p = .079) in the RPC blood supply regions, respectively. DAS28 was negatively correlated with CPD in RPC blood supply region (Rho = -0.272, p = .006). CONCLUSION: In RA, retinal CPD in the macula is lower than HCs. Although retinal CPD is not generally different in active and inactive RA patients, capillaries in the optic disc may be affected by disease activity.

Optical coherence tomography angiography findings in axial spondylarthritis.

The aim of this study is to evaluate the density of retinal vascular structures and their relationship with disease duration and activity in patients with axial spondylarthritis (axSpA) using optical coherence tomography angiography (OCT-A). In this case-control study, 56 eyes of 29 axSpA patients and 61 eyes of 31 healthy controls (HCs) were evaluated using OCT-A. The capillary plexus density (CPD) of vessels in the parafovea and perifovea regions was evaluated from the superficial capillary plexus (SCP) and deep capillary plexus (DPC) flow areas. The CPD of vessels in the fundus was evaluated from the radial peripapillary capillary (RPC) flow area. Foveal avascular zone (FAZ) size was measured. In axSpA patients and HCs, the mean CPD (%) in the whole retina was 50.75 ± 2.59 and 52.43 ± 2.10 (p < 0.001) in SCP, 54.00 ± 5.83 and 58.37 ± 4.36 (p < 0.001) in DCP, and 50.13 ± 2.32 and 50.13 ± 2.26 (p = 0.984) in RPC flow areas, respectively, and the mean FAZ (mm) was 0.275 ± 0.10 and 0.294 ± 0.07 (p = 0.281), respectively. A significant negative correlation was detected between the CPD and disease duration in the parafovea (ρ: - 0.306, p = 0.022), parafovea superior-hemi (ρ = - 0299, p = 0.025), parafovea inferior-hemi (ρ = - 0.270, p = 0.044), parafovea temporal (ρ = - 0.349, p = 0.008) and parafovea nasal regions (ρ = - 0.356, p = 0.007) in the DCP flow area. CPD was found to be lower in the macula, while similar in the fundus region in axSpA patients compared to HCs. The decrease in CPD was correlated with disease duration, but not with disease activity in axSpA. Small vessel structures may be affected in axSpA. OCT-A may be used to detect subclinical vasculitis in axSpA patients.

The relationship between CRP gene polymorphism (rs2794521, rs3091244), ASDAS-CRP and ASDAS-ESR in ankylosing spondylitis.

Background: We aimed to investigate the haplotypes and alleles of two variants (rs2794521 and rs3091244) in AS patients and to examine their relationship with ASDAS-CRP and ASDAS-ESR values.Methods: We evaluated 160 AS patients diagnosed according to the ASAS criteria. ASDAS-CRP and ASDAS-ESR values were calculated. ESR and CRP were examined. The restriction fragment length polymorphism (RFLP) method was used for detecting the rs2794521 and rs3091244 regions on the CRP gene.Results: As a result of the evaluation of rs2794521 gene polymorphism using PCR, TT, TC and CC genotypes were observed in 90, 81 and 9 individuals, respectively. As a result of the evaluation of rs3091244 gene polymorphism, CC, AC and TT genotypes were observed in 104, 51 and 5 individuals, respectively. T allele and C allele were found in rs2794521 gene by 75% and 25%, respectively. In addition, T allele, C allele and A allele were found in rs3091244 gene by 80%, 17% and 3%, respectively. With the help of regression equation, ASDAS-CRP level was 0.34 units higher in cases with rs3091244 C allele than cases without rs3091244 C alleles.Conclusion: CRP rs3091244 C allele may be associated with the increased relative risk for ASDAS-CRP.

Parameters of arterial stiffness in patients with Behçet's disease and their relationship with disease duration.

The results of investigations of arterial stiffness in Behçet's disease (BD) are contradictory and the reason for this contradictory situation is not clear. The lack of studies in homogenous groups according to the duration of the disease may be the cause of conflicting results. To compare arterial stiffness by assessing pulse wave velocity (PWV) and augmentation index (AIx) measurements in healthy controls (HC) and patients diagnosed with BD with short and long disease duration. This cross-sectional study was conducted between August-November 2017 and 54 patients with BD and 34 HC were included. Patients with BD who were diagnosed within 12 months were included in the group with short disease duration (SDD) and the others in the group with long disease duration (LDD). Parameters of cardiovascular risk of all participants were recorded and PWV and AIx values were measured from the brachial artery. AIx was significantly higher in all patients with BD, patients with BD with SDD and patients with BD with LDD, than in HC (p = 0.005, p = 0.011, p = 0.004, respectively). Pulse wave velocity values were not different from HC in patients with BD. When patients with BD with SDD and LDD were compared with each other, PWV was significantly higher in patients with BD with LDD (p = 0.030). There was a moderate correlation between PWV and disease duration (Rho = 0.414, p = 0.002). Augmentation index is higher in patients with BD than HC regardless of disease duration.

Depression is the most significant independent predictor of fatigue in patients with primary Sjögren's syndrome.

Objectives: The study aimed to evaluate the level of fatigue and the relationship between mood, pain, fibromyalgia, insomnia, disease activity, and dryness with fatigue in primary Sjögren's syndrome (PSS) patients. Patients and methods: In this case-control study, the participants were recruited between January 2021 and July 2021. Functional Assessment of Chronic Illness Therapy Fatigue (FACIT-F), pain DETECT questionnaire, Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Insomnia Severity Index (ISI) were administered to 50 PSS patients (48 females, 2 males; mean age: 48.9±10.8 years; median age: 47 years; range, 29 to 71 years) and 60 healthy controls (HCs; 57 females, 3 males; mean age: 49.8±8.4 years, median age: 52 years; range, 32 to 72 years). In addition, EULAR Sjögren's syndrome disease activity index (ESSPRI), EULAR Sjögren's Syndrome Patient Reported Index (ESSDAI), pain thresholds, Schirmer tests, and whole unstimulated salivary flow rate measurements were determined in PSS patients. Independent predictors of fatigue (fatigue subscale scores <30.5) were investigated by logistic regression analysis. Results: The frequency of fatigue in PSS patients and HCs was 54.0% and 8.3%, respectively. The rates of mood disturbance (BDI ≥11) in PSS patients with and without fatigue were 70.4% and 13.1%, respectively. BDI (Rho=-0.804), BAI (Rho=-0.586), ISI (Rho=-0.483), and ESSDAI (Rho=-0.345) were negatively correlated with the fatigue subscale score. Depression [Odds ratio (OR): 1.214, confidence interval (CI): 1.007-1.463], fibromyalgia (OR: 21.674, CI: 1.470-319.469), disease activity (OR: 1.440; CI: 1.005-2.065), and insomnia (OR: 1.223, CI: 1.003-1.4922) were identified as independent predictors of fatigue in PSS patients. It was determined that BD alone could predict fatigue by 84% in PSS patients. Conclusion: Depression can be a prominent predictor of fatigue in PSS patients. There is a need for studies evaluating the effect of antidepressant treatment approaches on fatigue accompanied by mood disturbance in PSS patients.

The evaluation of the role of BMI and insulin resistance on inflammatory markers, PAI-1 levels and arterial stiffness in newly diagnosed type 2 diabetes mellitus patients.

BACKGROUND: Increased cardiovascular risk, represented by endothelial inflammation, probably starts with the very first course of type-2 diabetes (T2DM). Almost 85.2% of all T2DM patients are overweight or obese. Thrombosis accounts 80% of all deaths in patients with diabetes. The thrombotic-fibrinolytic equilibrium shifts in favor of thrombosis by plasminogen activator inhibitor-1 (PAI-1). PAI-1 secretion is induced primarily by CRP. PAI-1 overexpression predisposes unstable plaque development. The contribution of obesity and diabetes to this process is not clearly understood. In this study, we aimed to investigate comparison of inflammatory markers, PAI-1 levels and arterial stiffness according to BMI and impaired glucose metabolism in patient with newly diagnosed T2DM. METHODS: Newly diagnosed 60 T2DM patients were enrolled. Demographics and measurements were noted. Liver (AST, ALT), kidney (urea, creatinine, albumin/creatinine ratio), metabolic (fasting blood glucose, post-prandial blood glucose, insulin, c-peptide, HbA1c, total cholesterol, low-density lipoprotein [LDL], high-density lipoprotein [HDL], triglyceride) parameters, inflammatory markers [hsCRP, fibrinogen]), PAI-1 levels and pulse wave velocity was measured from all participants. The results were compared. RESULTS: Inflammatory markers and PAI-1 levels were significantly elevated in obese group compared to overweight participants. The correlation analysis showed that waist and hip circumferences, high-sensitive CRP, fibrinogen and PAI-1 levels were positively correlated with BMI but not with HbA1c levels. CONCLUSIONS: The results of our study showed that lipid levels, glycemic and blood pressure values of the obese and overweight patients were similar. BMI affects inflammatory markers and PAI-1 levels independent of glucose regulation and insulin resistance in newly diagnosed T2DM. According to the current study BMI is found to be more prominent in terms of inflammatory markers and PAI-1 levels compared to insulin resistance and impaired glucose metabolism in newly diagnosed T2DM.

The Frequency of Fibromyalgia and its Relationship With Disease Activity in Female Patients With Behçet's Disease: A Cross Sectional Study.

OBJECTIVES: This study aims to determine the incidence of fibromyalgia (FM) in female patients with Behçet's disease (BD) and to investigate whether disease activity changes in the presence of FM in female patients with BD. PATIENTS AND METHODS: This cross-sectional study included 72 female patients with BD (mean age 39.7±10.5 years; range, 22 to 74 years) between June 2018 and February 2019. The frequency of FM was evaluated according to the American College of Rheumatology alternative criteria. Active clinical features and BD activity were evaluated with the Behçet's disease activity form (BDCAF). RESULTS: The prevalence of FM was 29.2% in the BD patients. BD clinical activity index, patients' perceptions of disease activity and clinicians' overall perceptions of disease activity scores were significantly higher in BD patients with FM (p=0.003, p<0.001 and p=0.025, respectively). The active clinical features of BD were not different between the groups except for genital ulcer. The prevalence of active genital ulcers was higher in BD patients with FM than in BD patients without FM (38.1% vs. 13.7%, respectively). CONCLUSION: Disease activity is higher in female BD patients with FM than those without FM. High disease activity in female BD patients with FM may be associated with the presence of genital ulcers.