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Introduction: Patients with chronic inflammatory rheumatic diseases (CIRD) who receive intravenous therapy requiring hospitalization are likely to be more affected than those with receiving oral therapy during COVID-19 pandemic. We aimed to investigate the effect of the COVID-19 pandemic on adherence to treatment in patients with CIRD receiving intravenous treatments.

Background: Primary Sjögren's syndrome (pSS) is a disease associated with the overexpression of proinflammatory cytokines, and oxidative stress is one of the factors responsible for its etiopathogenesis. This study aimed to investigate the thiol/disulphide homeostasis in pSS patients. Methods: The study included 68 pSS patients and 69 healthy controls. Thiol/disulphide homeostasis (total thiol, native thiol, and disulphide levels) was measured using the automatic spectrophotometric method developed by Erel and Neselioglu, and the results of the 2 groups were compared. Results: The gender and age distributions of the pSS and control groups were similar (P = 0.988 and P = 0.065). Total thiol and native thiol levels were lower in the pSS group than in the control group (470.08 ± 33.65 mmol/L vs. 528.21 ± 44.99 mmol/L, P < 0.001, and 439.14 ± 30.67 mmol/L vs. 497.56 ± 46.70 mmol/L, P < 0.001, respectively). There were no differences in disulphide levels between groups 17.00 (range 0.70-217.0) mmol/L vs. 14.95 (range 2.10-40.10) mmol/L, P = 0.195. Conclusions: It was concluded that the thiol/disulphide balance shifted towards disulphide in patients with pSS.

Abstract Background High-throughput molecular interaction data have been used effectively to prioritize candidate genes that are linked to a disease, based on the observation that the products of genes associated with similar diseases are likely to interact with each other heavily in a network of protein-protein interactions (PPIs). An important challenge for these applications, however, is the incomplete and noisy nature of PPI data. Information flow based methods alleviate these problems to a certain extent, by considering indirect interactions and multiplicity of paths. Results We demonstrate that existing methods are likely to favor highly connected genes, making prioritization sensitive to the skewed degree distribution of PPI networks, as well as ascertainment bias in available interaction and disease association data. Motivated by this observation, we propose several statistical adjustment methods to account for the degree distribution of known disease and candidate genes, using a PPI network with associated confidence scores for interactions. We show that the proposed methods can detect loosely connected disease genes that are missed by existing approaches, however, this improvement might come at the price of more false negatives for highly connected genes. Consequently, we develop a suite called DADA, which includes different uniform prioritization methods that effectively integrate existing approaches with the proposed statistical adjustment strategies. Comprehensive experimental results on the Online Mendelian Inheritance in Man (OMIM) database show that DADA outperforms existing methods in prioritizing candidate disease genes. Conclusions These results demonstrate the importance of employing accurate statistical models and associated adjustment methods in network-based disease gene prioritization, as well as other network-based functional inference applications. DADA is implemented in Matlab and is freely available at http://compbio.case.edu/dada/.

Abstract Background In systems biology, comparative analyses of molecular interactions across diverse species indicate that conservation and divergence of networks can be used to understand functional evolution from a systems perspective. A key characteristic of these networks is their modularity, which contributes significantly to their robustness, as well as adaptability. Consequently, analysis of modular network structures from a phylogenetic perspective may be useful in understanding the emergence, conservation, and diversification of functional modularity. Results In this paper, we propose a phylogenetic framework for analyzing network modules, with applications that extend well beyond network-based phylogeny reconstruction. Our approach is based on identification of modular network components from each network separately, followed by projection of these modules onto the networks of other species to compare different networks. Subsequently, we use the conservation of various modules in each network to assess the similarity between different networks. Compared to traditional methods that rely on topological comparisons, our approach has key advantages in (i) avoiding intractable graph comparison problems in comparative network analysis, (ii) accounting for noise and missing data through flexible treatment of network conservation, and (iii) providing insights on the evolution of biological systems through investigation of the evolutionary trajectories of network modules. We test our method, MOPHY, on synthetic data generated by simulation of network evolution, as well as existing protein-protein interaction data for seven diverse species. Comprehensive experimental results show that MOPHY is promising in reconstructing evolutionary histories of extant networks based on conservation of modularity, it is highly robust to noise, and outperforms existing methods that quantify network similarity in terms of conservation of network topology. Conclusion These results establish modularity and network proximity as useful features in comparative network analysis and motivate detailed studies of the evolutionary histories of network modules.

Objectives Gender impact on phenotypical expression of Behçet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD. Methods Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behçet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout disease course, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated. Results In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean ± SD BODI (1.92 ± 2.09) at the last follow-up, compared to female patients (1.25 ± 1.87) (p<0.0001). Uveitis (p<0.0001) and vascular involvement (p=0.0076) were significantly more frequent among males whereas female patients were significantly overrepresented in arthralgia (p<0.0001), arthritis (p=0.00025), isolated headache (p<0.0001), central nervous system (CNS) involvement (p=0.040), and gastrointestinal involvement (p=0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (p=0.617). Four variables were associated with the development of major organ involvement: male gender (OR=2.104, p=0.001), current treatment with biologic agents (OR=2.257, p=0.0003), origin from endemic countries (OR=2.661, p=0.0009), and disease duration (OR=1.002, p=0.024). Conclusion BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Objectives: The aim of this study is to investigate the characteristics of Primary Sjögren's syndrome (pSS)- interstitial lung disease (ILD) patients and compare them to those of pSS patients without ILD in the tertiary pSS-ILD cohort to evaluate potential risk factors for ILD occurrence and disease progression., Methods: Patients followed up who met the 2016 American College of Rheumatology-European League Against Rheumatism classification criteria for pSS were retrospectively analyzed. The patients were grouped as those with ILD and those without ILD according to medical records. High-resolution computed tomography (HRCT)/ thorax CT (TCT) results of all ILD patients were evaluated. Data on demographics, comorbidities, clinical characteristics and laboratory findings were collected., Results: A total of 378 pSS patients, including 60 with ILD and 318 without ILD were detected to have at least one obtainable HRCT/TCT and were included in the study. In the cohort of pSS patients with at least one HRCT or TCT, the frequency of ILD was 15.8%. In the ILD group, the most common HRCT pattern was NSIP, and the most common findings were ground glass opacities, traction bronchiectasis, and honeycombing. Logistic regression analysis showed that male gender (OR:2.90), being diagnosed with pSS over the age of 50(OR:4,24), smoking history (OR:2.38), elevated LDH(OR:3.27), elevated ESR(OR:2.51) and lymphopenia (OR:5.12) were related with development of ILD while being diagnosed with ILD after the age of 60 (OR:8.5) was related with radiographic progression., Conclusion: The study results provided a large spectrum view for pSS-ILD and pointed out several risk factors for ILD occurrence and radiographic progression.

Introduction: Obese rheumatoid arthritis (RA) patients often show reduced responses to traditional treatments, including TNF inhibitors (TNFi). Considering the different mechanisms of action it is important to evaluate the efficacy of tofacitinib in obese patients. This study aims to explore the impact of obesity on the drug survival of tofacitinib in RA patients., Material and Methods: This retrospective cohort study included RA patients treated with tofacitinib. Patients were categorized into obese (BMI ≥ 30 kg/m 2 ) and non-obese (BMI < 30 kg/m 2 ) groups. The primary outcome was drug survival, assessed using Kaplan-Meier and logistic regression analyses., Results: The study comprised 80 RA patients, with 31 (39%) classified as obese. At the 12-month mark, the drug survival rate for tofacitinib was higher in the obese group (81%) compared to the non-obese group (59%). Contrary to univariable analysis, multivariate analysis did not identify obesity as a significant predictor of drug survival. Other variables including sex, hypertension, diabetes mellitus, and anti-cyclic citrullinated peptide (anti-CCP) positivity also showed no significant association with tofacitinib drug survival., Conclusion: The findings indicate that obesity does not alter the drug survival rate for tofacitinib among RA patients. Univariate analysis reported a potentially higher drug survival rate in obese patients; however, the lack of statistical significance in multivariate analysis and the study's retrospective nature necessitate further research to validate these observations and guide personalized therapeutic strategies for this population., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Objective: This study was designed to compare thiol/disulfide and ischemia-modified albumin (IMA) levels between psoriatic arthritis (PsA) and healthy controls and evaluate the correlation between these molecules and the disease activity scores used in PsA., Methods: A total of 63 PsA patients and 49 healthy volunteers were included in the study. Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), modified disease activity score 28 (DAS28), and Bath Ankylosing Spondylitis Functional Index (BASFI) scores were used as disease activity indices for PsA patients. Calculations of native thiol (-SH), disulfide (-SS), and total thiol (-SH+-SS) molecules were made by the automatic spectrophotometric method, and the albumin cobalt binding test was used to measure IMA levels., Results: In the PsA group, -SS/-SH and -SS/(-SH+-SS) levels were higher and -SH/(-SH+-SS) levels were lower than in controls. In the linear regression analysis, a significant correlation relationship was detected between DAS28-erythrocyte sedimentation rate (ESR) and -SS/(-SH+-SS) (β = 0.795, CI 95%, 0.196-1.395; P = .010), -SH/(-SH+-SS) (β = -0.475, CI 95%, 0.114-0.836; P = .010) and IMA (β = 3.932, CI 95%, 0.859-7.005; P = .013). Additionally, a significant correlation was detected between IMA and BASDAI and BASFI., Conclusion: In PsA, thiol/disulfide homeostasis has shifted in favor of disulfide as an oxidative indicator. Serum thiol/disulfide levels are correlated with PsA disease activity indices., (© The Author(s) 2024. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Recent data show that netrin-1 has a role in development of pulmonary fibrosis. This study was aimed to investigate serum netrin-1 level and its relation to interstitial lung disease(ILD) in patients with rheumatoid arthritis (RA)., Method: 42 RA patients with RA-ILD, 58 RA patients without RA-ILD (RA non-ILD group), and 61 healthy volunteers were included in this study. The modified DAS28-ESR score was used to calculate disease activity in RA patients. Using the quantitative immunoassay method, Serum netrin-1 levels were measured with an ELISA kit (Catalog number: E-EL-H2328; lab science, lot number: GZWTKZ5SWK, Texas, USA)., Results: The median value of netrin-1 was found to be significantly higher in the RA-ILD group (82.9 [59.9-124]) compared to both the RA non-ILD group(52.9 [49.5-73.1])(B = -0.006, OR = 0.994, CI 95 %=0.989-0.999, P = 0.018) and the control group(53.5 [49.5-87.5]) (B: -0.005, OR: 0.994, CI 95 %: 0.990-0.999, p: 0.022). A cut-off value of 61.78 for netrin-1 was found to have a sensitivity of 73.8 % and a specificity of 69 % for the diagnosis of RA-ILD (AUC [95 %Cl] = 0.771 [0.679-0.862], p < 0.0001).It was found that high serum netrin-1 level was strongly associated with the RA-usual interstitial pneumonia(UIP) pattern and poorly related to the RA-nonspecific interstitial pneumonia(NSIP) pattern compared to the RA non-ILD group., Conclusions: Netrin-1 is elevated in the serum of patients with RA-ILD, especially in the UIP pattern. Netrin-1 may be a potential candidate for predicting the development of RA-ILD that should be investigated in the pathophysiological and therapeutic fields.., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

Aim: Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disease in the world. There are known triggers to initiate an FMF attack, yet potential effects of intrauterine devices (IUD) in women of reproductive age have not been evaluated before., Method: Consecutive female patients with FMF who ever used IUD over the age of 18 were enrolled. Female patients with FMF were sub grouped according to the type of IUD they use. FMF attack frequency, severity, duration, presence of dysmenorrhea, severity of dysmenorrhea, having attacks during menstruation before and after IUD use were questioned. Demographic and clinical data were collected from hospital database., Results: When all patients with IUD use were evaluated, it was found that the frequency of attacks increased after IUD insertion at 3rd and 12th months (median [min-max] attack frequency at 3rd month, 1 (0-3) vs 1 (0-6), p = 0.002, median [min-max] attack frequency at 12th month, 2 (0-12) vs 3.5 (0-18), p = 0.028). Attack severity measured by VAS pain was also significantly increased. Attack duration and menstrual pain was similar before and after IUD use. Attack frequency at 3rd and 12th months, attack severity and menstrual pain was all increased significantly in Cu-IUD users, whereas none of these parameters deteriorated in LNG-IUD group., Conclusion: IUD use, especially Cu-IUD, may increase the frequency and severity of attacks in female patients with FMF. Clinicians may benefit from considering LGN-IUD if IUDs are preferred as contraception in women of childbearing age with FMF., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by persistent antiphospholipid antibodies (aPLs) with arterial and venous thrombosis and/or pregnancy morbidity. In recent years, several studies have highlighted the potential role of non-criteria aPL in diagnosing APS patients., Aim: This study aimed to determine the association of the presence of non-criteria aPL antibodies to the clinical and laboratory features of patients with a diagnosis of APS., Methods: Eighty patients diagnosed with APS and under observation in the rheumatology clinic of Ankara City Hospital were assessed. Patient demographic and clinical features were meticulously recorded. Non-criteria antibodies tested in our center included antiphosphatidylserine IgA, antiphosphatidylserine IgM, beta 2 glycoprotein IgA, anti-cardiolipin IgA, antiphospholipid antibody IgG, and antiphospholipid antibody IgM. Antibodies from patients who were tested for at least one non-criteria antibody were documented., Results: Out of 80 patients, 55 (68.8%) were tested for at least one non-criteria antibody, and 29 of those patients (52.7%) tested positive for at least one non-criteria antibody. The antiphospholipid antibody IgM and the beta 2 glycoprotein IgA were the most commonly tested non-criteria antibodies. Patients with non-criteria antibody positivity had a higher frequency of Ds DNA positivity and low complement (62.0% vs. 35.0%, p = 0.042; 69.0% vs. 38.0%, p = 0.023), respectively. In addition, positivity for anti-cardiolipin IgG and b2 glycoprotein IgG was significantly higher in the group positive for non-criteria antibodies (79% vs. 31%, p ≤ 0.001; 72.0% vs. 19%, p ≤ 0.001). There was no significant difference between the clinical features of patients with at least one positivity for non-criteria antibodies and those without., Conclusion: Systemic lupus erythematosus (SLE) is the most commonly associated disease with APS, being present in approximately 35% of cases [1]. Since the majority of the patient group in our study had APS that was secondary to SLE, non-criteria antibody positivity may be linked to the immunological activity of SLE. Large multicenter studies are necessary to investigate the clinical significance of isolated/combined positivity for criterion/non-criteria aPLs., (© 2023. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

This study investigated the possible roles of renal estrogen receptors (ER) in glomerulonephritis associated with small vessel vasculitis. The relationships of ERs were investigated in antineutrophilic cytoplasmic antibody (ANCA)-associated glomerulonephritis and immunoglobulin A (IgA) nephropathy groups, which are small vessel vasculitis subtypes with two different glomerulonephritis development pathophysiologies. The design of this study was prepared as a retrospective cohort study. The study included 42 patients with ANCA-associated vasculitis and 18 with IgA nephropathy in the small vessel vasculitis group. For the control group, intact renal tissues of 28 patients who underwent nephrectomy due to renal cell carcinoma were used. Renal biopsy samples of the groups were stained with ER beta (ß) and ER alpha (α). Tubular ER ß expression score (TERßES) median values were found to be significantly higher in ANCA- associated vasculitis (B = 0.724, OR [95%CI]: 2.064 [1.141-3.731], p = .016) and IgA nephropathy (B = 0.898, OR [95%CI]: 2.454 [1.307-4.609], p = .005) than in intact kidney tissue. It was determined that tubular ERß was most frequently localized in the distal tubule at 57.9% and the second most common in the proximal tubule at 20.4%. The expression of tubular ERß is increased in glomerulonephritis due to small vessel vasculitis. Tubular ERßs are most commonly localized in the distal tubule. Further studies are needed to understand the physiological and pathophysiological effects of altered renal ER levels in small vessel vasculitis., (© 2024 The Authors. The FASEB Journal published by Wiley Periodicals LLC on behalf of Federation of American Societies for Experimental Biology.)

Aim: The clinical symptoms and laboratory markers of Rheumatoid Arthritis (RA) and Psoriatic Arthritis (PsA) can be very similar, so making a differential diagnosis between these two diseases is often difficult. Serological parameters to be used in differential diagnosis can guide the clinician. This study aimed to investigate the usability of 14-3-3η (eta) protein as a biomarker in the differential diagnosis of PsA and RA, and the relationships between eta protein and disease activity scores and joint erosions in PsA and RA., Methods: 54 PsA patients, 53 RA patients, and 56 healthy individuals were included in this study. The ELISA (Enzyme-Linked ImunoSorbent Assay) kit was used as a quantitative sandwich enzyme immunoassay technique to detect human eta protein levels. Receiver- operating Characteristic (ROC) curves analysis was used to determine the sensitivity and specificity of the eta protein., Results: Eta protein levels were found to be significantly higher in the RA group than in the PsA [B: -0.341, OR (95% CI): 0.711 (0.556-0.909), p: 0.007] and control [B: -0.225, OR (95% CI): 0.798 (0.641-0.995), p: 0.045] groups. Eta protein median values were significantly higher in patients with joint erosion than in those without [β= 0.151, OR (95% CI): 1.163 (1.003-1.349), p: 0.046]., Conclusion: Eta protein levels are higher in the serum of RA patients than PsA and are associated with joint erosion. Eta protein may be a potential biomarker in the differential diagnosis of RA and PsA. It may represent a possible therapeutic step in the pathophysiological pathways in the development of joint erosion., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Introduction: Compared to biological agents, little is known about the impact of sulfasalazine therapy on COVID-19 outcomes in patients with Axial Spondyloarthritis (AxSpA). Therefore, we aimed to evaluate the COVID-19 severity in AxSpAs receiving sulfasalazine and biologic-agent., Materials and Methods: A total of 219 SARS-CoV-2 positive AxSpA patients were retrospectively analyzed. COVID-19 pneumonia, hospitalization rate, and length of stay were used to determine COVID-19 severity. AxSpA patients were mainly grouped and compared as sulfasalazine and non-sulfasalazine. Afterward, we excluded no-treatment patients to reveal the drug's effects more clearly and regrouped AxSpA patients as sulfasalazine-monotherapy (34.3%), biologic-monotherapy (33.7%), and sulfasalazine + biologic (7.3%)., Results: Fifty-nine percent of the patients were male and the mean age was 45.0 years. Peripheral arthritis was 35% and uveitis 15%. In total, 41.5% of them have received sulfasalazine and 41.0% biologic agents, and the remaining patients with no AxSpA-specific treatment. In the first comparison, the sulfasalazine group had a higher age, more frequent COVID-19 pneumonia, hospitalization, and longer hospitalization than a non-sulfasalazine group. In the pairwise comparison of 3 treatment groups, the demographic and clinical features, the hospitalization rate and the length of hospital stay were similar but the sulfasalazine-monotherapy group had a higher frequency of COVID-19 pneumonia than the biologic-monotherapy group (23% vs. 7%, p  = 0.008)., Conclusion: Our results imply sulfasalazine may be related to more severe COVID-19 in AxSpA patients. These patients should be followed more carefully in the presence of COVID-19, regardless of reasons such as age, comorbidity, and extra-axial disease, and consideration of discontinuing sulfasalazine maybe even thought.

Background: Estrogen has been thought to play an essential role in the disease pathogenesis of systemic lupus erythematosus, which is 9-10 times more prevalent in the female population. It has been shown that irregular estrogen/estrogen receptor signaling pathways may contribute to the pathophysiology of various renal diseases. In this study, we compared renal estrogen receptors between lupus nephritis, familial Mediterranean fever-associated renal amyloidosis, ANCA-associated nephritis, and intact kidney to investigate their role in the pathophysiology of renal diseases., Methods: This study was designed as a retrospective cohort study. Thirty systemic lupus erythematosus patients with lupus nephritis, 12 familial Mediterranean fever amyloidosis and 10 ANCA-associated glomerulonephrites, and 14 individuals with normal renal histology were included in the study., Results: Tubular estrogen receptor ß expression score was found to be significantly higher in the familial Mediterranean fever [5 (1-8)] group than in the lupus nephritis [0 (0-1)] (B = 1.385, OR = 3.996, CI %95 = 1.805-8.846, p = .001) and ANCA [4 (1-6.5)] (B = -1.431, OR = 0.239, CI 95% = 0.093-0.614, p = .003) groups. A significant correlation was found between serum creatinine values and tubular estrogen receptor ß expression score (OR = 0.565, CI 95% = 0.622-1.402, p < .0001). In ANCA-associated glomerulonephritis, a significant relationship was found between fibro cellular crescents in renal biopsy and glomerular estrogen receptor ß expression score (OR = 0.247, CI 95% = 0.11-0.999, p = .045) and tubular estrogen receptor ß expression score (OR = 0.282, CI 95% = -0.180-2.812, p = .026)., Conclusions: This study showed that tubular estrogen receptor ß expression score was elevated in familial Mediterranean fever amyloidosis and correlated with serum creatinine levels and renal crescents., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Primary Sjögren syndrome (PSS) is a chronic, autoimmune, and lymphoproliferative disease of the connective tissue. In patients with PSS, the risk of developing B-cell non-Hodgkin lymphoma (NHL) increases dramatically, with a prevalence of approximately 5%. The 14-3-3 protein isoforms are phospho-serin/phospho-threonine binding proteins associated with many malignant diseases. This study aimed to evaluate the relationship between disease activity parameters and markers predicting lymphoma development in patients with PSS and 14-3-3η proteins., Methods: This study was designed as an analytical case-control study. A total of 57 PSS patients and 54 healthy volunteers were included in the study. The European League Against Rheumatism (EULAR) Sjögren syndrome disease activity index (ESSDAI) was used to assess systemic disease activity in PSS. Receiver operating characteristic (ROC) analysis was used to test the diagnostic accuracy measures of the analytical results. Multivariable linear regression analysis was used to evaluate the effects of independent variables on the 14-3-3η protein., Results: The 14-3-3η protein serum levels were found to be significantly higher in PSS (2.72 [2.04-4.07]) than healthy controls (1.73 [1.41-2.43]) ( P <0.0001). A significant relationship was found between 14-3-3η protein levels and ESSDAI group (β=0.385, 95%CI=0.318-1.651, P =0.005), hypocomplementemia (C3 or C4) (β=0.223, 95% CI=0.09-1.983, P =0.048) and purpura (β=0.252, 95% CI=0.335-4.903, P =0.022), which are accepted as lymphoma predictors. A significant correlation was found between PSS disease activity score ESSDAI and 14-33η protein (β=0.496, 95% CI=0.079-0.244, P =0.0002)., Conclusion: 14-3-3η proteins are potential candidates for diagnostic marker, marker of disease activity, and predictor of lymphoma in PSS patients., (© 2023 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Objectives: To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect., Methods: Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry., Results: In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 ± 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy., Conclusions: Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflicts of interest for this work., (Copyright © 2023. Published by Elsevier Inc.)

BACKGROUND / AIM: The use of PET / CT is becoming more common in the elucidation of inflammatory processes in which the underlying cause cannot be determined by conventional examinations. Although PET / CT is an effective method for detecting inflammatory foci, the precise diagnosis may not be obtained in all cases. In addition, considering factors such as radiation exposure and cost, it becomes important to identify patients who can get results with PET / CT. In this study, it was aimed to examine the factors that can predict the differential diagnostic value of PET / CT by retrospectively scanning patients who underwent PET / CT for inflammation of unknown origin (IUO) in rheumatology practice., Methods: Demographic, clinical and laboratory information of the patients followed up in our clinic and who underwent PET / CT for differential diagnosis were enrolled. Whether they were diagnosed after PET / CT and during the follow - up period, and their diagnoses were examined., Results: A total of 132 patients were included in the study. A previous diagnosis of rheumatic disease was present in 28.8 % of the patients, and a history of malignancy was present in 2.3 % . The patients were divided into three groups: group 1 patients with increased FDG uptake in PET / CT and diagnosis confirmed by PET / CT, group 2 patients with increased FDG uptake in PET / CT but diagnosis was not confirmed, and group 3 patients without increased FDG uptake in PET / CT. Increased FDG uptake in PET / CT was detected in 73 % of the patients. While PET / CT helped the diagnosis in 47 (35.6 %) patients (group 1), it did not help the diagnosis in 85 (64.4 %) (groups 2 and 3). Thirty - one (65.9 %) of the diagnosed patients were diagnosed with a rheumatologic disease. When the 3 groups were compared, male gender, advanced age, CRP levels, presence of constitutional symptoms, SUVmax values and number of different organs with increased FDG uptake were higher in Group 1. Sixty - six percent and 74 % of the patients in groups 2 and 3 were not diagnosed during the follow - up period. No patient in group 3 was diagnosed with malignancy during follow - up., Conclusion: PET / CT has high diagnostic value when combined with clinical and laboratory data in the diagnosis of IUO. Our study revealed that various factors can affect the diagnostic value of PET / CT. Similar to the literature, the statistically significant difference in CRP levels shows that patients with high CRP levels are more likely to be diagnosed with an aetiology in PET / CT. Although detection of involvement in PET / CT is not always diagnostic, there was an important finding that no malignancy was detected in the follow - up in any patient without PET / CT involvement. Key points • PET / CT is an effective method for detecting inflammatory foci. • PET / CT has proven to be effective in the diagnosis of rheumatological diseases, the extent of disease and the evaluation of response to treatment. • Indications for the use of PET / CT in the field of rheumatology and the associated factors and clinical features supporting the diagnosis with PET / CT are still to be fully clarified. • In routine practice, with PET / CT, both delays in diagnosis and examinations performed during diagnosis and the cost can be reduced., (© 2023. International League of Associations for Rheumatology (ILAR).)

Background: The aim of this study is to determine the demographic, clinical and laboratory characteristics of the patients who followed up with the diagnosis of sarcoidosis, to investigate the distribution frequency of rheumatological findings and to examine the disease management from the perspective of rheumatology., Methods: Patients who were followed up with the diagnosis of sarcoidosis in the rheumatology clinic of Ankara City Hospital between November 2019 and November 2022 were evaluated. Demographic, clinical, radiological, serological, laboratory, and histopathological findings, and rheumatological, systemic, and locomotor system examination findings of the patients were obtained from the medical data registered in the hospital., Results: A total of seventy sarcoidosis patients (48.98 ± 11.78 years, %75 female) were included in the study. Joint involvement was observed in 64.3% of cases, skin involvement in 48.6% of cases, and ocular involvement in 25.7% of cases. The ankle was the most frequently involved joint, followed by the knee and small joints in the foot. Corticosteroids were the most used therapeutic agent, and pulmonary and joint findings were the most common reasons for starting treatment., Conclusions: Sarcoidosis is a disease that mimics many diseases, misdiagnosis and treatment should be avoided with a good and fast differential diagnosis. Clinicians, especially rheumatologists, should remember sarcoidosis more frequently and keep it in mind in the differential diagnosis.

Various studies reported that the kynurenine (Kyn) pathway plays a pivotal role in regulating the balance between activation and inhibition of the immune system. Proinflammatory cytokines can accelerate the Kyn pathway by altering indoleamine (2, 3)- dioxygenase (IDO) allosteric enzyme activity. Excessive cytokine release and immune system activation have essential roles in the pathogenesis of axial spondyloarthritis (axSpA). We aimed to investigate the relationship of the Kyn pathway with proinflammatory cytokines and with the severity of the disease in patients with axSpA. The study included 104 patients with axSpA and 54 healthy volunteers. The severity of the disease was determined by Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). The Kyn pathway was evaluated by IDO activity calculated with Kyn/Tryptophan (Trp) ratio. Plasma Trp and Kyn concentrations were measured with tandem mass spectrometry. Serum IL 17/23 and IFN-γ concentrations were measured with ELISA. These groups were compared in terms of IDO, IL-17, IL-23, IFN-γ, and BASDAI. Plasma IDO activity was significantly increased, however, serum IL-17, IL-23, and IFN-γ levels were significantly decreased in patients compared to healthy volunteers. While IFN-γ was positively correlated with the severity of the disease (p = 0.02), it also had a significant inverse correlation with IDO activity (p < 0.001). However, these correlations are weak. As a result of this study, the Kyn pathway is accelerated and proinflammatory cytokine levels are decreased in patients with axSpA. All of these results with an indirect weak negative association between high IDO and low disease activity suggest that an accelerated Kyn pathway may limit the immune system activation in axSpA disease., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Introduction: The aim of the study is to investigate serum levels of 14-3-3 η (ETA) protein in patients with gout and possible relations with joint damage., Method: This cross-sectional study included 43 gout patients and 30 control patients., Results: Serum 14-3-3 η protein levels were significantly higher in gout patients (median [IQR], 3.1 [2.0] vs 2.2 [1.0], p = 0.007). In subgroup analyses of gout patients, serum 14-3-3 η protein levels did not differ between patients with and without a flare, tophaceous disease, elevated CRP and serum uric acid levels and a history of chronic kidney disease; however, were significantly higher in the patients with erosions (Median [IQR], 4.1 [2.7] vs 2.7 [1.5], p = 0.002). According to ROC curve, serum 14-3-3 η protein had 86.0% sensitivity and 30% specifity at a cut-off point of 1.7 ng/mL and had 74.7% sensitivity and 43.3% specifity at a cut-off point of 2.0 ng/mL., Conclusion: Our results demonstrated elevated levels of 14-3-3 η protein in gout patients which is more prominent in patients with erosive changes, implying role of 14-3-3 η protein in inflammatory and structural damage related pathways and suggesting a potential as a marker for disease severity., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

Background: To determine the rate and types of neurological involvement in patients with primary Sjögren's syndrome (pSS) and to evaluate predictive clinical and immunologic features of neurological involvement., Methods: We retrospectively assessed 2127 patients with an ICD-10 code for Sjögren recorded in the hospital database. Among these patients, those meeting the pSS classification criteria and having neurological symptoms and an objective evaluation accordingly were enrolled. After comparing the patients with and without neurological involvement, peripheral and central involvement subtypes were also compared within themselves., Results: A total of 199 pSS patients were enrolled and neurological involvement was found in 31.6%. Peripheral nervous system (PNS) involvement was found in 23.5% of the patients, and central nervous system (CNS) involvement was found in 34.3%. Patients with neurological involvement had a higher frequency of Schirmer's test, anti-Ro/SS-A and anti-La/SS-B positivity and the presence of interstitial lung disease, articular involvement, lymphadenopathy, anemia and hypocomplementemia than patients without those. In multivariate regression analysis, only articular involvement had a higher risk for the development of neurologic involvement [OR 10.01 (4.18-23.97), P 0.0001]. Among the patients with PNS, the frequency of anti-Ro/SS-A positivity, low C3 and Schirmer's test positivity were statistically increased compared to those who were not in PNS ( P = 0.032, P = 0.044, and P = 0.029, respectively). When compared in terms of CNS involvement, patients with CNS involvement were younger, had a shorter disease duration, and had a higher frequency of anti-Ro/SS-A positivity than patients without those ( P = 0.041, P = 0.027, and P = 0.046, respectively)., Conclusions: In our study, it was shown that one third of the symptomatic pSS patients had objective neurological involvement. The presence of neurological symptoms should be considered, especially in patients with articular involvement in pSS., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Annals of Indian Academy of Neurology.)

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet's disease (BD)., Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry., Results: Respondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet's Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0-30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1-50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range - 1.8-4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status ( p  < 0.001), the presence of any major organ involvement ( p  < 0.031), the presence of gastro-intestinal ( p  < 0.001), neurological ( p  = 0.012) and musculoskeletal ( p  = 0.022) symptoms, recurrent fever ( p  = 0.002), and headache ( p  < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD ( F  = 14.519, OR 1.162 [CI 0.557-1.766], p  < 0.001)., Discussion: Preliminary results from the AIDA for Patients BD registry were consistent with data available in the literature, confirming that PROs and PREs could be easily provided by the patient remotely to integrate physician-driven registries with complementary and reliable information., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Gaggiano, Del Bianco, Sota, Gentileschi, Ruscitti, Giacomelli, Piga, Crisafulli, Monti, Emmi, De Paulis, Vitale, Tarsia, Caggiano, Nuzzolese, Parretti, Fabiani, Lopalco, Maier, Cattalini, Rigante, Govoni, Li Gobbi, Guiducci, Parronchi, Marino, Ciccia, Maggio, Aragona, Bartoloni, Iagnocco, Viapiana, Sebastiani, Guerriero, Insalaco, Del Giudice, Conti, Barone, Olivieri, Brucato, Carubbi, Triggianese, Mauro, Tosi, Fonollosa, Giardini, Ragab, Tharwat, Hernández-Rodríguez, Sfikakis, Laskari, Karamanakos, Espinosa, Shahram, Direskeneli, Hinojosa-Azaola, Opris-Belinski, AlMaghlouth, Hatemi, Eksin, Önen, Więsik-Szewczyk, Akkoç, Tufan, Şahin, Erten, Ozen, Batu, Frediani, Balistreri and Cantarini.)

Objectives: This study aimed to translate the Scleroderma Skin Patient-Reported Outcome (SSPRO) questionnaire to the Turkish (SSPRO-T) language and to assess its validity and reliability., Patients and Methods: Fifty-four systemic sclerosis (SSc) patients (51 females, 3 males; mean age: 49.8±10.4 years; range, 22 to 65 years) participated in the reliability and validity analysis between October 2022 and December 2022. The translation and cross-cultural adaptation of the SSPRO-T was applied in accordance with the procedure described by the Beaton guidelines. The SSPRO-T, the Scleroderma Health Assessment Questionnaire (SHAQ), the Health Assessment Questionnaire Disability Index (HAQ-DI), Skindex-29, and patient global skin severity were conducted in all participants for construct validity. The SSPRO-T was retested to assess its reliability after seven days., Results: The SSPRO-T had a four-factor structure. The total SSPRO-T score and its subgroups correlated positively with SHAQ, HAQ-DI, Skindex-29, and patient global skin severity. The internal consistency and reliability were excellent in overall SSPRO-T and in the subgroups: physical effect, emotional effect, physical limitation, and social effect (Cronbach's α=0.94, 0.80, 0.95, 0.93, and 0.84, respectively). The SSPRO-T had excellent test-retest reliability (r=0.91, p<0.001). In addition, no floor effect or ceiling effect was observed., Conclusion: The SSPRO-T questionnaire is a reliable and valid tool and can be used in research and clinical practice in Turkish patients with SSc., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish League Against Rheumatism.)

Introduction: Rituximab, which is used in autoimmune rheumatic diseases (ARD), can cause both an increased risk of development of COVID-19 disease and re-infection due to its potent and long-acting immunosuppression. So, we aimed to evaluate the frequency, risk factors and re-infection rates of COVID-19 in ARD patients receiving rituximab., Methods: A single-center retrospective study was performed with patients receiving rituximab for ARD in 12 months before the onset of COVID-19 in Turkey. The data regarding severe acute respiratory syndrome-coronavirus 2 reverse transcription polymerized chain reaction (RT-PCR) test, clinical, laboratory, and mortality data of all patients were collected from medical records. Logistic regression analysis was used for predictors of COVID-19 disease. COVID-19 re-infection was defined as RT-PCR positivity and recurrence of acute COVID-19 symptoms after at least 1 negative RT-PCR in patients with clinical improvement., Results: Ninety-eight ARD patients with rituximab were evaluated and 23 (23%) of them had COVID-19. The presence of hypogammaglobulinemia increased the risk of COVID-19 disease 8-fold. COVID-19 pneumonia occurred in 13 (57%) and these patients' age was higher than those without pneumonia (59.6 ± 11.8 vs 44.9 ± 14.2 years, P = 0.013). Mortality due to COVID-19 was 13% and COVID-19 re-infection was seen in 20% of survivors., Conclusion: Regardless of the underlying rheumatic disease and organ involvements, hypogammaglobulinemia in ARD could be a risk factor for COVID-19 development, and advanced age could be for COVID-19 severity. Moreover, COVID-19 re-infection rates are high., (© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Autoimmune rheumatic diseases have their own specific clinical presentation, and can affect multiple systems. Neurological involvement of autoimmune rheumatic diseases may involve both the central and peripheral nervous systems. Inflammation of neural tissue, autoantibody-mediated reactions, and small vessel vasculitis may be effective in the pathogenesis of neuropathy in autoimmune rheumatological diseases. Autoimmune rheumatic disease with pure motor neuron involvement is very rare in the literature. The case is here presented of a 58-year-old female patient who presented with the complaints of increasing pain and weakness in the extremities and was diagnosed with lower motor neuron disease and overlap syndrome. The patient was treated with cyclophosphamide, pulse steroid, hydroxychloroquine and intravenous immunoglobulin. After 3 months of treatment, a significant improvement was observed in the patient's clinical complaints and laboratory parameters. In conclusion, some patients with undiagnosed autoimmune rheumatic diseases may have neurological complaints. Clinicians should investigate patients with such neurological complaints for autoimmune rheumatic diseases., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: It has been suggested that the deterioration in the antioxidant defense system due to thiols may cause the pro-oxidant/antioxidant imbalance seen in rheumatoid arthritis (RA). This study was conducted to evaluate thiol/disulfide (-SH/-SS) homeostasis in patients with RA compared to healthy controls, and to validate the limited number of studies examining the relationship between Disease Activity Score-28 (DAS28) and thiol parameters., Method: A total of 100 individuals (mean age: 46.3 ± 12.03) consisting of 86 females and 14 males were included in the RA group, and a total of 100 individuals (mean age: 43.3 ± 10.96 years) consisting of 78 females and 22 males were included in the control group. DAS28 was used to assess RA disease activity. -SH/-SS homeostasis parameters were measured using the automated spectrophotometric method described by Erel and Neselioğlu., Results: While native thiol (-SH) (p:0.001), total thiol (-SH + -SS) (p < 0.0001) levels and -SH\(-SS + -SH) ratio (p: 0.018) were lower in the RA group compared to the healthy controls, disulfide (-SS) level (p: 0.005)), -SS\-SH (p: 0.001) and -SS\(-SS + -SH) (p: 0.002) ratios were found to be higher. In the control group and the group in remission (defined by DAS28 < 2.6), the median values of -SH (p:0.002) and -SS + -SH (p:0.0008) were found to be significantly higher, and the median value of -SS (p: 0.001) was found to be lower compared to the other DAS28 groups. While a negative correlation was found between DAS28 and -SH (r: -0.243, p: 0.007), a positive correlation was found between DAS28 and -SS (r: 0.316, p: 0.0003), -SS\-SH (r:0.229, p: 0.002) and -SS\(-SS + -SH) (r: 0.285, p: 0.0009)., Conclusions: The plasma thiol antioxidant pool was decreased in RA compared to healthy controls and those with active disease compared to those in remission., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

Aim: To determine frequency of adverse events and attacks related to vaccination in recipients of CoronaVac and BNT162b2 in familial Mediterranean fever (FMF) patients, and to search whether history of prior COVID-19 or a booster dose increases occurrence of adverse events/attacks., Methods: FMF patients were surveyed for administration of any COVID-19 vaccine and vaccine-related adverse events or FMF attacks. Demographic, clinical, vaccine-related data, history of COVID-19 infection before or after vaccination, adherence to FMF treatment during vaccination were collected., Results: A total of 161 vaccinated FMF patients were included. Ninety-three patients out of 161 had reported suffering from an adverse event/attack after a vaccine dose. There were 54.7% of BNT162b2 recipients who reported any adverse event after any vaccine dose in comparison to 29.9% of CoronaVac recipients (P < .001). There were 22.2% of BNT162b2 recipients who reported suffering from a FMF attack within 1 month after vaccination in comparison to 19.4% of CoronaVac recipients (P = .653). When patients with or without adverse event/attack were compared, no significant differences were observed in means of demographics, comorbid diseases, disease duration, total vaccine doses, or treatments adhered to for FMF. Rates of adverse events/attacks were similar between patients with and without prior COVID-19. In booster recipients, adverse events/attacks were most frequent after the booster dose., Conclusions: A considerable number of FMF patients suffered from vaccine-related adverse events/attacks, particularly with BNT162b2. No serious events or mortalities due to vaccination were detected. Demographics, clinical characteristics and prior history of vaccination did not significantly affect these results. We observed an increased rate of adverse events/attacks with booster dose administration., (© 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective: The aim of this study is to evaluate semaphorin 3A levels in patients with systemic lupus erythematosus (SLE) with and without renal involvement and secondary antiphospholipid antibody syndrome (APS)., Methods: Patients with SLE were grouped according to the presence of secondary APS or renal involvement. The control group consisted of age-matched, nonsmoking, healthy volunteers. Semaphorin 3A levels were compared among groups. All patients with SLE were regrouped according to the presence of thrombotic events, miscarriages, and proteinuria, and semaphorin 3A levels were investigated. Finally, semaphorin 3A levels of all patients with SLE as a single group were compared to those of the control patients., Results: The mean semaphorin 3A values were 16.16 ± 2.84 ng/mL in the control group, 9.05 ± 5.65 ng/mL in patients with SLE without nephritis and APS, 11.28 ± 5.23 ng/mL in the SLE with APS group, and 8.53 ± 5.11 ng/mL in the lupus nephritis group. When all 3 patient groups were examined as a single group, the mean semaphorin 3A value was significantly lower than that of the control group. Semaphorin 3A was reduced in patients with SLE with thromboembolism and/or history of miscarriage., Conclusion: Semaphorin 3A levels were lower in all patient groups compared to the control group. Moreover, the reduced semaphorin 3A levels in patients with a history of thromboembolism and/or miscarriage suggest that semaphorin 3A may play an important role in the pathogenesis of vasculopathy., (© American Society for Clinical Pathology, 2021. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: Rheumatologic diseases may impair the quality of life (QoL) by affecting sexual functions in different ways. We aimed to evaluate sexual functions and the disease-related variables, physical and psychogenic states in female patients with ankylosing spondylitis and non-radiographic axial spondyloarthropathy., Methods: A total of 98 women with axial spondyloarthropathy (axSpA) and 99 healthy females were included in the study. The axSpA group was divided into two subgroups as ankylosing spondylitis (AS) and non-radiographic axial spondyloarthropathy (nr-axSpA) (62 AS and 36 nr-axSpA). The patients' disease-related variables recorded. All the women in the axSpA and control groups were evaluated gynaecologically. The female sexual function index (FSFI), Health Status Questionnaire [Short Form (SF)-36], and Hospital Depression and Anxiety Scale (HADS) were applied to all participants., Results: Clitoral and labial atrophy and speculum pain score were significantly higher in the axSpA group (p<0.05). The FSFI and QoL-SF-36 scores were significantly lower and the HAD-D and HAD-A scores were significantly higher of in the axSpA group than in the control group (p<0.05 for all). There was no significant between the axSpA subgroups in terms of the FSFI, QoL-SF-36 and HAD scores., Conclusions: In elderly women with axSpA, disease duration and limitation of movement are more effective in genital atrophy and sexual functions, but there is no difference between those with AS and nr-axSpA in relation to sexual functions and psychological burden.

Objective: TURKBIO registry, established in 2011, is the first nationwide biological database in Turkey. This study aimed to provide an overview of TURKBIO data collected by June 2018., Methods: The registry included adult patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS), nonradiographic axial spondyloarthritis (nr-AxSpA), and psoriatic arthritis (PsA). Demographic and clinical features, disease activity markers, and other follow-up parameters, current and previous treat- ments, and adverse events were registered electronically at each visit using open-source software. The registration of patient-reported outcome measures was carried out electronically by the patients using touch screens., Results: TURKBIO registry included a total of 41,145 treatment series with biologicals. There were 2,588 patients with axSpA (2,459 AS and 129 nr-axSpA), 2,036 with RA, and 428 with PsA. The total number of patients, including those with other diagnoses, was 5,718. In the follow-up period, the number of patients and also visits steadily increased by years. The yearly mean number of visits per patient was found to be 2.3. Significant improvements in disease activity and health assessment parameters were observed following the biological treatments. Biologics were often given in combination with a con- ventional synthetic disease-modifying antirheumatic drug in patients with RA. Infections were the most commonly seen adverse events, followed by allergic reactions. Tuberculosis was observed in 12 patients, malignancy in 18, and treatment-related mortality in 31., Conclusion: TURKBIO provided a valuable real-life experience with the use of biologics in rheumatic diseases in Turkey.

Objectives: Sexual dysfunctions in patients with rheumatological diseases can negatively affect human sexual life, and thus lead to the deterioration of quality of life. This study aimed to determine the effects of primary Sjögren's syndrome (pSS) on female sexual organs and sexual functions., Methods: A total of 68 women with pSS and 135 healthy female patients were included in the study. All the women in the study and control groups were evaluated gynaecologically, and genital findings during the examination and variables related to pSS were recorded. The women's sexual functions were evaluated with the Female Sexual Function Index (FSFI) and quality of life was evaluated using the Health Status Questionnaire-Short Form 36 (QoL-SF 36)., Results: There was no difference in terms of the ages of the patients between the pSS and control groups [50 (25-70) and 49 (23-70) years, respectively] (p=0.487). The FSFI and QoL-SF 36 scores of the pSS group were significantly lower than the control group (p<0.05). Although the age of the patients, duration of menopause, and presence of atrophy on genital examination significantly correlated with sexual dysfunction, there was no significant correlation between pSS activity-related variables and sexual dysfunction., Conclusions: It was determined that pSS led to sexual dysfunction by causing genital atrophy and vaginal dryness in women. Moreover, mood changes associated with the disease, especially depression, were revealed to be an independent risk factor for this condition.

Objective: The aim of this study was to determine the frequency of sarcopenia at the time of diagnosis in patients with rheumatoid arthritis (RA), assessing disease activity and factors that may be associated with sarcopenia and observe effects of treatment on sarcopenia., Method: A prospective study was conducted with patients who have newly diagnosed RA. Patients were evaluated twice, at the time of diagnosis and 3 mo after the initiation of treatment. Demographic data, anthropometric measurements, disease activity scores, and sarcopenia status were recorded. Sarcopenia was evaluated with grip strength and bioelectric impedance. The results were compared with healthy volunteers., Results: The age at onset of RA was 50.6 ± 14.6 y. Handgrip strength (P < 0.001), skeletal muscle mass (P = 0.009), and skeletal muscle mass index (P = 0.032) were reduced in patients with RA but not in the control group. The frequency of sarcopenia in RA at onset of diagnosis was 31.5%. There was a significant decrease in the rate of sarcopenia after 3 mo of treatment (31.5 versus 8.7%; P = 0.046)., Conclusion: Sarcopenia was found in approximately one-third of the patients with newly diagnosed RA in our study. With treatment, sarcopenia improved significantly. Patients with RA should be evaluated in terms of sarcopenia in addition to evaluating joint and extraarticular findings at the time of diagnosis. Early detection and treatment planning may improve quality of life., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Aim: Autoantibody development plays an important role in the pathogenesis of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). In this study, we aimed to determine the diagnostic value of anticarbamylated protein antibody (anti-CarP) antibody in SLE and RA patients and its relationship with disease prognosis. Material & method: Fifty-seven SLE patients (F/M 50/7; median age 40.9 ± 13.7; median disease duration 2 years) who met the 2012 SLICC SLE diagnostic criteria were included in the study. A total of 46 RA patients selected according to the 2010 ACR/EULAR diagnostic criteria (F/M 38/8; median age 54.2 ± 12.4; median disease duration 2 years) were included. A total of 30 healthy individuals were selected as the control group. The anti-CarP antibody was studied by using human anticarbamylated protein antibody ELISA Kit (SunRedBio, Shanghai, China). Results: Anti-CarP antibody positivity was found to be 17.4% in RA patients (p < 0.001), 54.4% in SLE patients (p < 0.001) and 3.3% in the healthy control group. The anti-CarP antibody was determined to predict SLE patients with 54.4% sensitivity and 96.7% specificity compared with the healthy control group (area under the curve: 0.755; p < 0.001). Conclusion: Anti-CarP antibody positivity was significantly higher in the SLE patients compared with the healthy control and RA group. It has significant sensitivity and specificity in both SLE and RA patients compared with the healthy controls.

Objectives: To explore the impact of early versus late-onset psoriasis (PsO) on the disease characteristics of psoriatic arthritis (PsA) in a large-multicentre cohort., Methods: The data from a multicentre psoriatic arthritis database was analysed. Patients were grouped according to age at psoriasis onset (early onset; <40 years of age, late-onset; >40 years of age) and disease characteristics of the groups were compared by adjusting for BMI and PsA duration, where necessary., Results: At the time of analyses, 1634 patients were recruited [62.8% females; early onset 1108 (67.8%); late-onset, 526 (32.2%)]. The late-onset group was more over-weight [66.8% vs. 86.8%, p<0.001; adjusted for age - aOR 1.55 (1.11-2.20; 95% CI)]. The early onset group had more scalp psoriasis at onset (56.7% vs. 43.0%, p<0.001), whereas extremity lesions were more common in the late-onset group (63.8% vs. 74.2%, p<0.001). Axial disease in males and psoriatic disease family history in females were significantly higher in the early onset group [38.0% vs. 25.4%; p=0.005; adjusted for PsA duration - aOR 1.76 (1.19-2.62; 95% CI) / 39.5% vs. 30.1%; p=0.003; OR 1.51 (1.15-1.99; 95% CI), respectively]. Psoriatic disease activity parameters, patient-physician reported outcomes and HAQ-DI scores were similar in both groups., Conclusions: Clinical features of PsA may be affected by the age at onset of PsO. Different genetic backgrounds in early and late-onset PsO may be driving the differences in psoriasis and PsA phenotypes.

Aim: Seronegative spondyloarthropathies (SpA) are a group of chronic diseases, characterized by axial inflammation, oligoarthritis, and enthesitis. Oxidative stress may contribute to a wide range of diseases such as rheumatologic diseases including SpA. This prospective case-control study was designed to compare the thiol-disulfide levels as a marker of oxidative stress in SpA patients with healthy controls., Methods: A total of 144 patients who were diagnosed as undifferentiated spondyloarthropathy (UspA, n=97), ankylosing spondylitis (AS, n=47), and 80 healthy controls were included. Serum native thiol (NT), total thiol (TT), disulfide (D) levels were measured with the fully automated Erel?s method. NT/TT, D/TT, and D/NT ratios were calculated. Thiol-disulfide levels were compared between SpA groups and healthy controls., Results: NT and NT/TT ratios were found to be significantly lower in the SpA group. (p<0.001). Disulfide, D/NT, and D/TT ratios were found to be significantly higher in the SpA group (p <0.001 for each comparison). In pairwise comparisons between SpA subgroups, NT and TT levels were lower in USpA group compared to AS group (P=0.021). Serum disulfide levels were higher in USpA group compared to AS group (P=0,004). Anti-tumor necrosis factor (Anti-TNF) group had lower TT measurements compared to the classical disease-modifying anti-rheumatic drugs (cDMARD) group in patients with SpA (P=0.039)., Conclusion: Thiol-disulfide balance is disturbed in favor of disulfide in SpAs patients compared to healthy volunteers. Native and total thiol measurements correlate with acute phase reactants and might be used to monitor disease activity. Anti-TNF therapy might control the oxidative degenerative process better than the classical DMARD in SpA patients.

Background/aim: Seronegative spondyloarthropathies (SpA) are a group of chronic diseases characterized by axial inflammation, oligoarthritis, and enthesitis. Oxidative stress may contribute to a wide range of rheumatologic diseases, including SpA. This prospective case-control study was designed to compare thiol-disulfide levels as a marker of oxidative stress between SpA patients and healthy controls., Materials and Methods: A total of 144 patients diagnosed with undifferentiated spondyloarthropathy (USpA, n = 97) or ankylosing spondylitis (AS, n = 47) were included along with 80 healthy controls. Serum native thiol (NT), total thiol (TT), and disulfide (D) levels were measured using the fully automated Erel method. The ratios NT/TT, D/TT, and D/NT were calculated. Thiol-disulfide levels were compared between the SpA groups and the healthy controls., Results: The NT and NT/TT ratios were found to be significantly lower in the SpA group (p < 0.001). The disulfide, D/NT, and D/TT ratios were found to be significantly higher in the SpA group (p < 0.001). In pairwise comparisons between the SpA subgroups, the NT and TT levels were lower in the USpA group than in the AS group (p = 0.021), but serum disulfide levels were higher in the USpA group than in the AS group (p = 0.004). Among the patients with SpA, the group taking antitumor necrosis factor (anti-TNF) had lower TT measurements compared to the group taking conventional disease modifying antirheumatic drugs (DMARD) (p = 0.039)., Conclusion: The thiol-disulfide balance is disturbed in favor of disulfide in SpA patients compared to healthy volunteers. Native and total thiol measurements correlate with acute phase reactants and might be used to monitor disease activity. Anti-TNF therapy might control the oxidative degenerative process better than the conventional DMARD in SpA patients., Competing Interests: Conflict of interest statement: The authors declare that there is no conflict of interest. This study conforms to the Helsinki Declaration and was approved by the ethics review board of the Ankara Yıldırım Beyazıt University Faculty of Medicine., (© TÜBİTAK.)

Introduction: The risk of tuberculosis is higher in cases who have used antiTNF treatments. However, it is not clearly known whether there is a relationship between other biologic agents and the risk of developing tuberculosis or not. We aimed to investigate the prevalence of active tuberculosis among patients with rheumatic disease treated with biologic drugs., Materials and Methods: The study was performed at a tertiary referral center from January 2015 to December 2019. A total of 2000 patients with rheumatic diseases were screened and 461 patients were enrolled in the study due to regular records. They were underwent LTBI screening tests and were followedup at least 1 year after TNF inhibitor treatment initiation., Result: The median age of all patients was 48 (min-max: 19-80). 283 patients (61.3%) were female and 178 (38.7%) were male. The most common diseases were ankylosing spondylitis (67.2%), rheumatoid arthritis (26%) and psoriatic arthritis (5.2%). Anti-TNF treatments were given to 85.2% of all cases and other biologic treatments were given to 14.8%. Tuberculin skin test was applied to 429 patients and 70.4% positivity was found. Quantiferon-TB test was applied to 93 patients and 20.4% positivity was found. 320 patients were treated for LTBI due to positive tuberculin skin test and/or positive quantiferon-TB test. TB was developed in only one patient out of 393 patients who were treated with anti-TNF treatments and the the prevalence of TB development was found 255/100.000., Conclusions: The incidence of tuberculosis was quite low in our patients with rheumatic disease who were receiving anti-TNF treatment compared to previous studies. Also, in patients who were using other biological treatments, no TB cases were developed.

Background/aim: The distribution of Mediterranean fever (MEFV) gene mutations in Turkish familial Mediterranean fever (FMF) patients varies according to geographic area of Turkey. There is a need for highly representative data for Turkish FMF patients. The aim of our study was to investigate the distribution of the common MEFV mutations in Turkish FMF patients in a nationwide, multicenter study., Materials and Methods: Data of the 2246 FMF patients, from 15 adult rheumatology clinics located in different parts of the country, were evaluated retrospectively. The following mutations have been tested in all patients: M694V, M680I, M694I, V726A, and E148Q., Results: There were 1719 FMF patients with available genetic testing. According to the genotyping, homozygous M694V, present in 413 patients (24%), was the most common mutation . One hundred and fifty-four (9%) of patients had no detectable mutations. Allele frequencies of common mutations were: M694V (n = 1529, 44.5%), M680I (n = 423, 12.3%), V726A (n = 315, 9.2%), E148Q (n = 214, 1%), and M694I (n = 12, <1%)., Conclusion: In this large-scale multicenter study, we provided information about the frequencies of common MEFV gene mutations obtained from adult Turkish FMF patients. Nearly half of the patients were carrying at least one M694V mutations in their alleles.

Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF., Methods: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database., Results: Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group., Conclusion: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

Aim: To evaluate the sleep quality and the relation of sleep quality with depression, anxiety, fatigue and disease activity in adult patients with familial Mediterranean fever (FMF)., Method: One hundred and seventy-four FMF patients and 84 age-sex matched healthy individuals were included in this study. The Pittsburgh Sleep Quality Index (PSQI), Multidimensional Assessment of Fatigue (MAF) and the Hospital Anxiety and Depression Index (HADS) were used to assess sleep quality, fatigue, depression and anxiety, respectively., Result: FMF patients had significantly higher depression, anxiety, fatigue and PSQI scores than healthy controls. As the severity of the disease increased, scores of total PSQI and its domains increased. Patients with total PSQI score higher than 5 had statistically significantly higher erythrocyte sedimentation rates (ESR), serum C-reactive protein and serum amyloid levels during attacks, more attack numbers in last 3 months and worse fatigue, depression scores. Total PSQI score was positively correlated with inflammatory markers during attacks, attack numbers in the last 3 months and fatigue score. Logistic regression models identified disease duration, ESR during attacks, fatigue, attack numbers in the last 3 months as predictors of poor sleep quality., Conclusion: Poor sleep quality is common in adult FMF patients. Anxiety, depression and fatigue are more frequent in FMF patients than healthy individuals. Poor sleep quality is associated with inflammatory marker levels during attacks, fatigue and attack numbers in the last 3 months., (© 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)