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Background. Behçet's disease (BD) is a chronic inflammatory vasculitis affecting multiple organs. Uveitis is frequently seen in patients with BD, especially in Turkish population. Objectives. To investigate vascular endothelial growth factor (VEGF) gene polymorphisms along with the levels of VEGF and VEGF receptors in patients with Behçet's uveitis (BU). Materials and methods. Fifty-five BD-associated uveitis patients and 30 age-and sex-matched controls were included in this case-control study. The genotypes of the single nucleotide poymorphisms (SNPs): rs2010963 (+405G), rs3025039 (+936T) and rs699947 (-2598A) of the VEGF-A gene were determined using real-time polymerase chain reaction (RT-PCR) and serum levels of VEGF and VEGF receptors were measured using enzyme-linked immunosorbent assay (ELISA). Results. No associations of the VEGF gene polymorphisms were observed in BD uveitis patients, but arthritis was present in 53.3% of patients not possessing CT genotype in C3025039→T polymorphism (p = 0.024). Although there were no statistically significant differences in serum VEGF-A, VEGF-C and soluble vascular endothelial growth factor receptor-3 (sVEGFR-3) levels (p < 0.05), serum vascular endothelial growth factor receptor-1 (VEGFR-1) and sVEGFR-3 levels were significantly higher in the BD group (p < 0.001 and p = 0.001, respectively). In addition, VEGF-C/soluble vascular endothelial growth factor receptor-2 (sVEGFR-2) ratio was significantly higher (p < 0.001), while VEGF-A/VEGFR-1 and VEGF-C/sVEGFR-3 ratios were significantly lower (p < 0.001 and p = 0.033, respectively) in BD patients compared to controls. Also, VEGF-C/sVEGFR-3 (p = 0.024, r = 0.37) and VEGF-C/sVEGFR-2 (p = 0.020, r = 0.38) ratios were positively correlated with disease duration. Conclusions. The significant changes in sVEGFR-3 levels and VEGF-C/sVEGFR-3 ratio has shown that lymphangiogenesis processes might take place in the pathogenesis of BD uveitis, and these parameters can be important indicators of evaluation of BD patients with uveitis together with disease duration. [ABSTRACT FROM AUTHOR]

Background: The course of novel coronavirus disease 2019 (COVID-19) has been of special concern in patients with inflammatory rheumatic diseases (IRDs) due to the immune dysregulation that may be associated with these diseases and the medications used for IRDs, that may affect innate immune responses. Objective: In this cohort study, we aimed to report the disease characteristics and variables associated with COVID-19 outcome among Turkish patients with IRDs. Methods: Between April and June, 2020, 167 adult IRD patients with COVID-19 were registered from 31 centers in 14 cities in Turkey. Disease outcome was classified in 4 categories; (i) outpatient management, (ii) hospitalization without oxygen requirement, (iii) hospitalization with oxygen requirement, and (iv) intensive care unit (ICU) admission or death. Multivariable ordinal logistic regression analysis was conducted to determine variables associated with a worse outcome. Results: 165 patients (mean age: 50 ± 15.6 years, 58.2% female) were included. Twenty-four patients (14.5%) recovered under outpatient management, 141 (85.5%) were hospitalized, 49 (30%) required inpatient oxygen support, 22 (13%) were treated in the ICU (17 received invasive mechanic ventilation) and 16 (10%) died. Glucocorticoid use (OR: 4.53, 95%CI 1.65-12.76), chronic kidney disease (OR: 12.8, 95%CI 2.25-103.5), pulmonary disease (OR: 2.66, 95%CI 1.08-6.61) and obesity (OR: 3.7, 95%CI 1.01-13.87) were associated with a worse outcome. Biologic disease-modifying antirheumatic drugs (DMARDs) do not seem to affect COVID-19 outcome while conventional synthetic DMARDs may have a protective effect (OR: 0.36, 95%CI 0.17-0.75). Estimates for the associations between IRD diagnoses and outcome were inconclusive. Conclusions: Among IRD patients with COVID-19, comorbidities and glucocorticoid use were associated with a worse outcome, while biologic DMARDs do not seem to be associated with a worse outcome. [ABSTRACT FROM AUTHOR]

Objectives: Behçet's disease (BD) is a type of systemic vasculitis and inflammatory disease of unknown etiology, which is associated with fatigue and lower quality of life (QoL). The aim of this study was to assess the relationship between Behçet's Disease Current Activity Form (BDCAF) and Behçet's Disease Quality of Life (BDQoL), depression, anxiety and fatigue in Behçet's disease. Methods: We conducted a cross‐sectional study of 155 BD patients and 107 healthy subjects in a single center over a 1 year period. All participants completed the Multidimensional Assessment of Fatigue (MAF) questionnaire, and the Hospital Anxiety and Depression (HADS) scale. Disease activity in the BD patients was assessed using the BDCAF, and the physician's global assessment (PGA). BD patients also completed the BDQoL questionnaire. Results: There was no significant difference in age and gender between the groups. BD patients had significantly higher HADS‐anxiety (HADS‐A), HADS‐depression (HADS‐D) and MAF scores than the healthy control group (P < 0.05). BD patients with active disease had significantly higher MAF and HADS‐A scores compared to inactive BD patients (P < 0.05). MAF scores showed positive correlations with HADS‐A, HADS‐D, BDCAF and BDQoL. Conclusions: Fatigue and anxiety are common in clinically active BD patients compared with healthy control subjects and inactive BD patients. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to validate the Patient self-Assessment of Skin Thickness in Upper Limb questionnaire (PASTUL) in systemic sclerosis (SSc) and assess impact of skin involvement on health-related quality of life (HRQoL)., Methods: Participants were included in four UK centres. PASTUL specifies a grading of skin at 8 sites corresponding to the modified Rodnan Skin Score (mRSS). Construct validity was assessed by comparing PASTUL scores with mRSS. HRQoL was evaluated with EQ5D5L and Leeds SSc HRQoL questionnaires. Additionally, correlation between PASTUL and Scleroderma Skin Patient reported Outcome (SSPRO) was explored. Follow-up was 12 months., Results: In total, 196 participants were included, mean age was 56.4 years (SD 13.9), 80.6% female (n = 158), mean disease duration 11.9 years (SD 9.9), 110 (56.1%) had limited cutaneous (lcSSc) and 81 (41.3%) diffuse cutaneous SSc (dcSSc). PASTUL and upper limb mRSS were well correlated at baseline, 6 and 12 months (ICC = 0.67, 0.78 and 0.62, p< 0.001). Test-retest reliability was good (ICC = 0.83, p< 0.001). There was a stronger correlation between PASTUL and upper limb mRSS in dcSSc compared with lcSSc (0.69 vs 0.51, p< 0.001). In participants with early disease (< 4 years) PASTUL was moderately correlated with HRQoL (r = 0.53, p< 0.001), correlations were weaker in the whole group. Mean time to do the PASTUL self-assessment was 5.0 min (SD 3.7)., Conclusion: PASTUL is a feasible outcome tool that adds to assessments as SSPRO. Skin thickening is correlated with HRQoL, particularly in early disease., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Because of concerns about malignancy risks, using biological disease-modifying antirheumatic drugs (bDMARDs) in patients with a history of malignancy remains a challenging issue in rheumatology practice. This study aimed to investigate bDMARD preferences of physicians when treating of rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients with a history of malignancy., Methods: The data for this cross-sectional study were gathered from the TReasure database using a date range of December 2017 and January 2020. Biological disease-modifying antirheumatic drug preferences were analyzed for 40 RA patients and 25 SpA patients with a history of malignancy., Results: The most frequently prescribed bDMARD was rituximab, which was given to 28 RA patients (70%). For 25 patients (62.5%), the time between the diagnosis of malignancy and starting on a bDMARD regimen was less than 60 months, with a median interval of 43.5 months. Among SpA patients, the preferred bDMARDs were secukinumab and etanercept, which were each administered to 7 patients (28%). For 13 SpA patients (52%), the time between the diagnosis of malignancy and starting on bDMARDs was less than 60 months, with a median interval of 97 months., Conclusions: The observed bDMARD preferences may be related to the therapeutic effects of rituximab on lymphoproliferative malignancies, the protective effects of secukinumab on tumor progression, and the short half-life of etanercept. Biological disease-modifying antirheumatic drugs should be used in RA and SpA patients with malignancy in case of high inflammatory activity., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Sarcopenia is a progressive and generalized loss of muscle mass and function. The aim of this study was to evaluate the frequency of sarcopenia among patients with primary Sjögren's syndrome (SS) and the factors related with sarcopenia., Methods: Forty-four female patients with primary SS and 44 female control subjects were included in this cross-sectional study between February and August 2019. Sarcopenia was evaluated by the handgrip test, Skeletal Muscle Mass Index, and gait speed test., Results: Eleven patients (25.0%) had presarcopenia in the SS group and 2 (4.5%) in the control group (p = 0.007). Compared with control subjects, SS patients had lower results of hand grip and gait speed tests (p = 0.005 and p < 0.001, respectively). According to the Mini Nutritional Assessment Short Form, patients with presarcopenia had higher risk of malnutrition compared with patients without sarcopenia (p = 0.043). Patients with presarcopenia had higher scores in the European League Against Rheumatism Sjögren's Syndrome Patient-Reported Index pain domain and patient visual analog scale for global disease activity compared with patients without sarcopenia (p = 0.044 and p = 0.036, respectively). In multivariate regression analysis, European League Against Rheumatism Sjögren's Syndrome Patient-Reported Index pain was associated with hand grip strength (p = 0.016, R2 = 0.13) and Mini Nutritional Assessment Short Form was associated with Skeletal Muscle Mass Index (p = 0.005)., Conclusions: Risk of sarcopenia is increased in patients with SS. Pain and malnutrition may contribute to presarcopenia. Evaluating pain and patient's global disease activity may help physicians to determine patients with increased risk of sarcopenia. Controlling disease activity and pain and preventing malnutrition may reduce the risk of development of sarcopenia., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Aim: Interleukin-6 receptor antagonist, tocilizumab (TCZ), is known to be effective in the treatment of amyloidosis in patients with familial Mediterranean fever (FMF). But there are limited data about the effect of TCZ on frequency of attacks. In the current study, we aimed to find out whether TCZ therapy could decrease the frequency of recurrent attacks of FMF or not., Materials and Methods: The recorded files of 15 patients who had received intravenous TCZ for the improvement of amyloidosis associated with FMF, were evaluated retrospectively. Data of demographic and clinical characteristics of patients were archived from those files., Results: Three female and 12 male patients received TCZ due to amyloidosis were included to the study. The mean age was 42.07 ± 14.37 years. All of the patients were in full compliance with colchicine treatment. According to international severity scoring system for FMF, all of the patients had severe disease. The frequency of attacks recorded was evaluated during TCZ treatment, and it was reported that one patient had no response, six patients had decreased attack frequency and eight patients had no attacks., Discussion: Tocilizumab is found to be efficient on improvement of amyloidosis and decreasing the frequency of recurrent attacks in patients with FMF. Besides, TCZ is well tolerated among the patients. Further and prospective studies with larger sample are needed to support these results.

Objectives: Immunoglobulin (Ig) A vasculitis affects children more commonly than adults and previous literature lacks any formal damage assessment. Our aim in this study is to investigate the disease course, relapse rates and prognostic factors in adult patients with IgA vasculitis and to evaluate the disease-related damage., Methods: We assembled a retrospective cohort of adult IgA vasculitis from six tertiary Rheumatology Centres in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records., Results: The study included 130 (male/female: 85/45) patients and the mean age was 42.2±17 years. Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations. One hundred thirteen patients (86.9%) were treated with oral glucocorticoids (GC). As additional immunosuppressive (IS) agents, azathioprine was given to 44 (34.9%) and pulse cyclophosphamide to 18 (12.6%) patients. Seventy-nine patients (60%) had follow-up of median 15 (IQR 7-40) months. Twelve (15%) patients relapsed during follow-up. The mean VDI score was 0.4 in the last visit. Nineteen (24.7%) patients had at least one damage item at the end of follow-up. Most frequent damage items were renal 11 (42%), ocular 4 (15%) and cardiovascular 4 (15%)., Conclusions: In this cohort the most frequent damage item was renal and was related to the disease itself. Damage score was higher in patients with more severe disease and treated more aggressively. Our results suggest that more effective treatment options are needed in a subgroup of patients with IgA vasculitis to prevent the damage related with the vasculitis, especially with more severe disease.

Introduction: Behçet's disease (BD) is a relapsing systemic inflammatory disorder. The diagnosis of BD is primarily based on clinical findings. Current biomarkers are not yet sufficient to diagnose and cannot anticipate the course of the disease and response to treatment. The aim of this study was to evaluate the relationship between the thiol-disulfide balance and disease activity and organ involvement in BD., Material and Methods: A hundred fifty patients with BD and 100 age- and gender-matched healthy controls were included in the study. Disease activity was assessed with the BD Current Activity form score. Serum levels of native thiol (NT), total thiol (TT), and disulfide were measured and the disulfide/native thiol, disulfide/total thiol and native thiol/total thiol levels were calculated for the patient and control groups., Results: Native thiol, total thiol, native thiol/total thiol values of the BD patients were significantly lower than those of the control group. The disulfide/native thiol, disulfide/total thiol values of BD patients were higher compared to the control group and the disulfide value of the BD group was slightly higher compared to the control group. No correlation was determined between thiol levels and disease activity and organ involvement in BD., Conclusions: In patients with Behcet's disease, the thiol-disulfide homeostasis balance shifted towards disulfide formation due to thiol oxidation. It may be used as a novel marker in BD because it is easy, practical, fully automated and relatively inexpensive., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2019 Termedia & Banach.)

Introduction: Behçet's disease (BD) is a complex multisystemic inflammatory disorder which is characterized by recurrent attacks of acute inflammation. As there is no universally recognized pathognomonic laboratory marker of BD, its diagnosis is still based on clinical findings., Aim: To evaluate the role of calprotectin and ischemia modified albumin (IMA) as biomarkers in the assessment of disease activity of BD., Material and Methods: A total of 93 patients with BD and 62 age- and gender-matched healthy controls were included in the study. Disease activity was assessed with the BD Current Activity Form (BDCAF) score. Serum levels of calprotectin, high-sensitivity C-reactive protein (hsCRP) and IMA were measured in the patient and control groups., Results: Serum levels of calprotectin, IMA and hsCRP in patients with BD were higher than those of the healthy control group ( p < 0.001 for all). No correlations between calprotectin and IMA, hsCRP, erythrocyte sedimentation rate, CRP, or BDCAF score were found., Conclusions: As the calprotectin level are increased in BD patients, it could be a candidate biomarker which plays a role in BD pathogenesis.