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Background Primary Sjogren’s syndrome is a chronic inflammatory autoimmune disease. Minor salivary gland biopsy is the gold standard for the diagnosis of primary Sjogren’s syndrome. Superb microvascular imaging, power Doppler ultrasound, and color Doppler of the salivary glands represent non-invasive, non-irradiating modality for evaluating the vascularity of the salivary glands in the diagnosis and follow-up of primary Sjogren’s syndrome. Aims To evaluate the efficacy of superb microvascular imaging and vascularity index in salivary glands for the sonographic diagnosis of primary Sjogren’s syndrome. Study design Prospective case-control study. Methods Twenty participants with primary Sjogren’s syndrome and 20 healthy subjects were included in the study. Both parotid glands and submandibular glands were evaluated by superb microvascular imaging, power Doppler ultrasound, and color Doppler. The diagnostic accuracy of superb microvascular imaging was compared using these techniques. Results In the patient group, the vascularity index values of superb microvascular imaging in parotid glands and submandibular glands were 3.5±1.66, 5.06±1.94, respectively. While the same values were 1.0±0.98 and 2.44±1.34 in the control group (p≤0.001). In the patient group, the vascularity index values of power Doppler ultrasound in parotid glands and submandibular glands were 1.3±1.20 and 2.59±1.82, respectively. While the same values were 0.3±0.32 and 0.85±0.68 in the control group (p≤0.001). The superb microvascular imaging vascularity index cut-off value for the diagnosis of primary Sjogren’s syndrome in parotid glands that maximizes the accuracy was 1.85 (area under the curve: 0.906; 95% confidence interval: 0.844, 0.968), and its sensitivity and specificity were 87.5% and 72.5%, respectively. While the superb microvascular imaging vascularity index cut-off value for the diagnosis of primary Sjogren’s syndrome in submandibular gland that maximizes the accuracy was 3.35 (area under the curve: 0.873; 95% confidence interval: 0.800, 0.946), its sensitivity and specificity were 82.5% and 70%, respectively. Conclusion Superb microvascular imaging with high reproducibility of the vascularity index has a higher sensitivity and specificity than the power Doppler ultrasound in the diagnosis of primary Sjogren’s syndrome. It can be a noninvasive technique in the diagnosis of primary Sjogren’s syndrome when used with clinical, laboratory and other imaging methods.

This study aimed to compare Tuberculin Skin Test (TST) and QuantiFERON (R)-TB Gold In-Tube (QFT-GIT) test in rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients scheduled for biological and targeted synthetic disease modifying anti-rheumatic drugs (DMARDs) in a Bacillus Calmette-Guerin-vaccinated population. Adult RA (n = 206) and SpA (n = 392) patients from the TReasure database who had both TST and QFT-GIT prior to initiation of biological and targeted synthetic DMARDs were included in the study. Demographic and disease characteristics along with pre-biologic DMARD and steroid use were recorded. The distribution of TST and performance with respect to QFT-GIT were compared between RA and SpA groups. Pre-biologic conventional DMARD and steroid use was higher in the RA group. TST positivity rates were 44.2% in RA and 69.1% in SpA for a 5 mm cutoff (p < 0.001). Only 8.9% and 15% of the patients with RA and SpA, respectively, tested positive by QFT-GIT. The two tests poorly agreed in both groups at a TST cutoff of 5 mm and increasing the TST cutoff only slightly increased the agreement. Among age, sex, education and smoking status, pre-biologic steroid and conventional DMARD use, disease group, and QFT-GIT positivity, which were associated with a 5 mm or higher TST, only disease group (SpA) and QFT-GIT positivity remained significant in multiple logistic regression. TST positivity was more pronounced in SpA compared to that in RA and this was not explainable by pre-biologic DMARD and steroid use. The agreement of TST with QFT-GIT was poor in both groups. Using a 5 mm TST cutoff for both diseases could result in overestimating LTBI in SpA.

Clustering is an important clinical feature of Behçet’s syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS phenotype differ substantially in terms of methodology. Correlation matrices and factor analyses were not efficient enough to uncover clusters. Clustering patterns may change according to demographic factors such as age and sex. Clustering patterns may also be profoundly influenced by the misperception of symptoms that are assumed to be secondary to BS, when, in fact, they represent manifestations of BD mimics. This can give rise to misleading conclusions and should be kept in mind when interpreting data obtained by clustering or other phenotype analyses of BS. A true geographical/racial variability in disease expression could be studied in a multinational consensus cohort. Pathogenetic studies in separate clusters of BS have still been lacking.

This study aimed to investigate the benefit of changing the pharmaceutical preparation of colchicine in Turkish Familial Mediterranean Fever (FMF) patients resistant to one preparation in terms of frequency of the attacks. Turkish adult FMF patients under treatment with an imported colchicine preparation—in the form of compressed tablet form—due to resistance to domestic colchicine preparations, which are film- or sugar-coated tablets, and not using anti-interleukin-1 or other biologic agents were included in the study. Baseline disease characteristics along with MEFV mutations were identified. Daily colchicine doses and attack frequencies before and after the pharmaceutical change were compared. Fifty patients resistant to coated tablet preparations of colchicine and under treatment with the compressed tablets were identified. The median duration of disease was 6 (interquartile range 2.7–14) years and duration under treatment with the imported colchicine was 21 (range 8–60) months. Eight (16%), ten (20%), and 32 (64%) patients had 0–3, 4–6, and more than 7 attacks per year, respectively, before the compressed tablets. After treatment with the compressed tablet form of colchicine, 44 (88%), 5 (10%), and 1 (2%) patients had 0–3, 4–6, and more than 7 attacks, respectively (p

Background: Several societies published recommendations for latent tuberculosis infection (LTBI) screening before biological and targeted synthetic disease-modifying anti-rheumatic drugs (DMARDs) in rheumatoid arthritis (RA) but not for other inflammatory arthritides such as spondyloarthritis (SpA). Using RA guidelines could result in overestimating Tuberculin Skin Test (TST) positivity in SpA. This study aimed to compare the distribution of TST results in SpA and RA patients along with comparison in terms of QuantiFERON®-TB Gold In-Tube (QFT-GIT) test in a Bacillus Calmette-Guérin-vaccinated population.Methods: Adult RA (n=206) and SpA (n=392) patients from the TReasure database who had both TST and QFT-GIT prior to initiation of biological and targeted synthetic DMARDs were included in the study. Demographic and disease characteristics along with pre-biologic DMARD and steroid use were recorded. The distribution of TST and performance with respect to QFT-GIT were compared between RA and SpA groups.Results: Pre-biologic conventional DMARD and steroid use was higher in the RA group. TST positivity rates were 44.2% in RA and 69.1% in SpA for a 5 mm cut-off (pConclusions: TST positivity was more pronounced in SpA compared to that in RA and this was not explainable by pre-biologic DMARD and steroid use. The agreement of TST with QFT-GIT was poor in both groups. Using a 5 mm TST cut-off for both diseases could result in overestimating LTBI in SpA.

Psoriatic arthritis (PsA) is an underdiagnosed entity with a broad impact on the quality of life. Although the pathogenesis is largely unknown, autoimmune footprints of the inflammation in PsA have increasingly been recognized. Most of the genetic variation predisposing to PsA is mapped to the class I major histocompatibility complex (MHC) region and shared by a variety of autoimmune diseases. Polymorphisms in the genes IL12B, IL23R, IL13, TNIP1, TRAF3IP2, TYK2, and many others explain the non- HLA genetic risk with little known functional consequences. Entheseal and synovial cellular infiltrate with oligoclonal CD8+ T cells and occasional germinal centers, loss of regulatory T cell function, and specific autoantibodies such as anti-PsA peptide, anti-LL-37, and anti-ADAMTSL5 are the immunopathological findings suggestive of autoimmunity. These were supported by clinical observations of autoimmune multimorbidity and treatment response to calcineurin/mTOR and co-stimulation inhibition.

Spondyloarthritis (SpA) is a group of diseases with overlapping skeletal and extra-articular features. Acute anterior uveitis (AAU) is the most common extra-articular manifestation of SpA. The relation between AAU and SpA is well defined in the current literature. Our study aims to analyze the frequency and factors associated with AAU in different forms of SpA in a large nationwide cohort of Turkish SpA patients.Retrospective cohort study.The data were obtained from the TReasure database, which compiles data from records of the web-based Rheumatoid Arthritis (RA) and SpA patients treated with biological disease-modifying anti-rheumatismal drugs from different regions of Turkey. The clinical characteristics of SpA and uveitis are recorded.Data of the 4,297 SpA patients were included in the study. Overall, 475 of 4,297 patients (11.0%) had experienced 1 or more episodes of uveitis. SpA patients with older age (P.001), a smoking history (P = .004), delayed diagnosis (P = .001), longer disease duration (P.001), arthritis (P.001), positive HLA-B27 (P.001), a family history of SpA (P.001), and radiographic damage (presence of sacroiliitis, syndesmophytes, bamboo spine, hip involvement) (P.001 for all) more commonly had uveitis. On the other hand, uveitis was less prevalent in patients with psoriasis and psoriatic arthritis (P.001 for both).Uveitis may be the key feature leading to SpA diagnosis. Patients with radiographic damage and long disease duration have an increased risk for uveitis in both male and female SpA patients. Patients with uveitis should be referred to a rheumatologist for a thorough evaluation of SpA.

Aim: This study aimed to explore loneliness and associated factors in Turkish patients with inflammatory arthritis. Method: Adult patients with rheumatoid arthritis (RA) (n = 58), ankylosing spondylitis (AS) (n = 53), and psoriatic arthritis (PsA) (n = 30), respectively, were included in the study. A single-item visual analog scale (VAS) for loneliness, UCLA Loneliness Scale-8 (ULS-8), Beck depression inventory (BDI), Beck anxiety inventory (BAI), revised multidimensional scale of perceived social support, Health Assessment Questionnaire-Disability Index (HAQ-DI) were used for the psychometric and functional assessments. Multiple regression models were generated for predicting the ULS-8 and HAQ-DI scores. Results: There was no difference between disease groups in terms of the ULS-8 and HAQ-DI scores. Among demographic and clinical parameters, only the education status and number of drugs used had associations with the ULS-8 score. Single-item VAS score for loneliness did not predict the ULS-8 score well. There were significant correlations between the ULS-8 and HAQ-DI, depression, anxiety, social support, and physician global VAS scores. Only the education status significantly predicted (β = -0.208) the ULS-8 score in multiple regression analysis (adjusted R2 = 0.15, P < .001). Beck depression, anxiety, and patient global VAS scores remained significant for predicting the HAQ-DI after multiple regression with the covariates ULS-8, depression, anxiety, social support, patient and physician global VAS scores, and the number of drugs used (adjusted R2 = 0.53, P < .001). Disease activity and the ULS-8 scores were not found to be associated in any disease group. Conclusion: Loneliness is associated with depression, anxiety, lack of social support, disability, higher number of drugs used, and lower education but not with disease activity in Turkish patients with RA, AS, and PsA. Perception and expression of loneliness vary according to the cultural background. Single-item scales for loneliness may lack reliability compared to the more comprehensive ULS-8. Keywords: ankylosing spondylitis; inflammatory arthritis; loneliness; psoriatic arthritis; rheumatoid arthritis; social support.

This study was undertaken to evaluate the diagnostic and prognostic values of pentraxin‐3 (PTX‐3) in patients with infected diabetic foot ulcers (IDFU) as well as to assess the association between PTX‐3 levels and IDFU severity. This study included 60 IDFU patients (Group 1), 45 diabetic patients without DFU (Group 2), and 45 healthy controls. Patients with IDFU were divided into mild, moderate, and severe subgroups based on classification of clinical severity. Patients who underwent amputation were also documented. Blood samples were collected to determine PTX‐3 levels. PTX‐3 levels in healthy controls, Group 1, and Group 2 were 5.83 (3.41‐20) ng/mL, 1.47 (0.61‐15.13) ng/mL, and 3.26 (0.67‐20) ng/mL, respectively. A negative correlation between plasma PTX‐3 and glucose levels was found. There were significant differences in terms of procalcitonin (PCT) and PTX‐3 levels in the subgroup analysis of Group 1. The PTX‐3 level in patients who did or did not undergo amputation was 4.1 (0.8‐13.7) and 1 (0.6‐15.1) ng/mL, respectively. Results suggest that PTX‐3 is a particularly effective marker in patients with IDFU, both in terms of predicting disease severity and assisting in the decision to perform amputation.

Antiphospholipid syndrome is a cause of arterial and venous thrombosis especially in the young adult population. Although dural sinus thrombosis is a relatively rare complication of antiphospholipid syndrome, it may be a cause of morbidity and mortality. Extension of thrombosis and involvement of deep venous structures are poor prognostic factors in patients with dural sinus thrombosis, but the rate of near-complete recanalization is not known in antiphospholipid syndrome-related dural sinus thrombosis. Herein, a case of antiphospholipid syndrome with multiple dural sinus, deep cerebral vein and internal jugular vein thromboses is presented with demonstrative imaging findings and near-complete recanalization after warfarin.

Angiotensin II type 1 receptor autoantibodies (AT1R-AAs) are known to be associated with malignant hypertension, preeclampsia, and vascular rejection in kidney transplantation. They were also suspected to have pathogenetic role in vasculopathic changes in systemic sclerosis (SSc). Clinical data regarding AT1R-AAs in SSc are scarce. In this work, we will examine the relationship between serum levels of AT1R-AAs and disease manifestations. Serum samples from SSc patients and healthy controls were analyzed for AT1R-AAs by using a commercial ELISA kit. We examined the association of serum levels of AT1R-AA with disease duration, systolic pulmonary artery pressure (sPAP) measurements, and disease manifestations like cutaneous, lung and esophageal involvements, and the presence of digital ulcers in a cross-sectional manner. There was no statistically significant difference in levels of AT1R-AAs between SSc (n = 93) patients and healthy controls (n = 66) (p = 0.23). Serum levels of AT1R-AAs were not correlated with disease duration, sPAP measurements, and showed no association with disease manifestations like lung involvement, esophageal involvement, digital ulcers, and cutaneous fibrosis. In our SSc cohort, AT1R-AA serum levels were not different from healthy subjects and higher levels were not associated with any disease manifestation neither.

Background: Articular manifestations may differ in ulcerative colitis (UC) and Crohn’s disease (CD). Genetic and non-genetic factors like sex, smoking, and presence of HLA-B27 were previously shown to modify the expression of articular and other extraintestinal manifestations of IBD. Objectives: The aim of this study is to document disease features and factors affecting the expression of articular manifestations in Turkish patients with IBD-related (enteropathic) arthritis under treatment with disease modifying antirheumatic drugs (DMARDs). Methods: Data regarding enteropathic arthritis (EA) were collected from the TReasure database, a nation-wide multicenter observational registry of inflammatory arthritis patients. Results: Among 4066 patients with seronegative spondyloarthropaties (SpA), 156 (3.8%) had EA, not reflecting a true prevalence due to selection bias. Demographic and clinical features according to IBD groups were summarized in Table 1. Rates of presence of sacroiliitis were similar between patients with UC and CD (39.9% and 60.1%, p=0.086 respectively). Rates of HLA-B27 positivity were 31.6% and 7.1% in patients with and without radiographic sacroiliitis, respectively (p=0.101). Enthesitis, dactylitis, psoriasis, family history forSpA, ESR, CRP, BASDAI and aSDAS levels had similar distributions in patients with and without radiographic sacroiliitis. Rates of “never-smoked” (26.5% vs 64.7%) and “current smoking” (32.4% vs 17.6%) significantly differed in patients with and without sacroiliitis (overall p=0.012) Conclusion: Our data confirm an association between smoking status and disease manifestations, particularly radiographic sacroiliitis. References [1] Fries W. Clinical features and epidemiology of spondyloarthritides associated with inflammatory bowel disease. World J Gastroenterol2009; 15: 2449-2455. Disclosure of interests: Orhan Kucuksahin: None declared, abdulsamet Erden: None declared, Ufuk Ilgen: None declared, Sedat Kiraz: None declared, ali Ihsan Ertenli: None declared, Nazife Sule Yasar Bilge: None declared, Timucin Kasifoglu: None declared, Ediz Dalkilic Grant/research support from: MSD and abbvie, Consultant for: MSD, abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, abbvie,Roche, UCB, Pfizer and Novartis, Cemal Bes: None declared, Nilufer alpay Kanitez: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, abbvie,Celltrion, Novartis, Pamir atagunduz: None declared, Belkis Nihan Seniz: None declared, Burcu Yagiz: None declared, Suleyman Serdar Koca: None declared, Muhammet Cinar: None declared, askin ates: None declared, Servet akar Grant/research support from: MSD, abbvie, Roche, UCB, Novartis, Pfizer, amgen, Consultant for: MSD, abbvie, Roche, UCB, Novartis, Pfizer, amgen, Speakers bureau: Pfizer, Onay Gercik: None declared, Duygu Ersozlu: None declared, Veli yazisiz: None declared, Gezmis Kimyon: None declared, Muge aydin: None declared, Ridvan Mercan: None declared, Burak Oz: None declared, Zeynel abidin akar: None declared, Omer Karadag: None declared, Bahar Kelesoglu: None declared, Sedat Yilmaz: None declared, Yavuz Pehlivan: None declared, Ender Terzioglu: None declared, Levent Kilic: None declared, Sukran Erten: None declared, Koray Tascilar: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, abbvie, Roche, UCB, Novartis, Pfizer and abdi Ibrahim, Speakers bureau: MSD, abbvie, Roche, UCB, Novartis, Pfizer and abdi Ibrahim

Background: In rheumatoid arthritis (RA), biologic DMARDs are important treatment options in resistant patients. Inefficacy or side effects may cause switching between these drugs. Objectives: This study aimed to determine features of patients switching from one biologic DMARD to another in RA treatment and to investigate associated reasons for switching. Methods: This multicenter, prospective observational cohort study used the TReasure database in which web-based registration of RA and spondyloarthritis patients are being performed in 15 centers across different regions of Turkey. In this study, data of RA patients switching from one biologic agent to another were analyzed. Demographic and clinical data, follow-up duration, time to switch, and reasons for switching were retrieved from the database. Results: Of the included 2115 RA patients, 829 (39.2%) switched between biologic agents (switched group) and 1286 (60.8%) continued to receive their current therapies (continued group). The median follow-up duration of all patients was 3.7 years and the median time to switch was 1.1 years. In the switched group, the proportion of females and the median HAQ-DI score were higher as well as disease duration was longer (Table 1). Among the biologic agents used at first, 60.9% of the patients were receiving an anti-TNF agent and 39.1% of the patients were receiving other biologic agents (Table 2, figure 1). In the switched group (n=829), the main reasons for switching were secondary inefficacy (n=269), primary inefficacy (n=238), and side effects (n=178) followed by primary or secondary unknown inefficacy (n=30), patient’s demand (n=21), physician’s request (n=16), willing to be pregnant (n=7), other (n=31), and unknown (n=54). Conclusion: The patients in the Treasure database were followed-up approximately 4 years and about one-third of the patients had to switch from one biologic DMARD to another. The main reasons for this switching were primary (29.2%) and secondary (33.0%) inefficacy and 20% of the patients had to switch due to side effects. According to the switching pattern, about half of the patients using an anti-TNF agent at first switched to another anti-TNF agent and the other half switched to other biologic agents. Disclosure of Interests: Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Ali Ihsan Ertenli: None declared, Abdulsamet Erden: None declared, Orhan Kucuksahin: None declared, Timucin Kasifoglu: None declared, Ediz Dalkilic Grant/research support from: MSD and Abbvie, Consultant for: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Cemal Bes: None declared, Nilufer Alpay Kanitez: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, Abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, Abbvie,Celltrion, Novartis, Pamir Atagunduz: None declared, Belkis Nihan Coskun: None declared, Burcu Yagiz: None declared, Suleyman Serdar Koca: None declared, Muhammet Cinar: None declared, Askin Ates: None declared, Servet Akar Grant/research support from: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Speakers bureau: Pfizer, Onay Gercik: None declared, Duygu Ersozlu: None declared, Veli Yazisiz: None declared, Gezmis Kimyon: None declared, Muge Aydin: None declared, Ridvan Mercan: None declared, Burak Oz: None declared, Nazife Sule Yasar Bilge: None declared, Zeynel Abidin Akar: None declared, Omer Karadag: None declared, Ayse Bahar Kelesoglu Dincer: None declared, Sedat Yilmaz: None declared, Ufuk Ilgen: None declared, Yavuz Pehlivan: None declared, Ender Terzioglu: None declared, Levent Kilic: None declared, Sukran Erten: None declared, Sedat Kiraz: None declared

Background In spondyloarthritis (SpA), biologic DMARDs are important treatment options in resistant patients. Inefficacy or side effects may cause switching between these drugs. Objectives This study aimed to determine features of patients switching from one biologic agent to another in SpA treatment and to investigate associated reasons. Methods This multicenter, prospective observational cohort study used the TReasure database in which web-based registration of rheumatoid arthritis and SpA patients are being performed in 15 centers across different regions of Turkey. In this study, data of SpA patients switching from one biologic agent to another were analyzed. Demographic and clinical data, follow-up duration, time to switch, and reasons for switching were retrieved from the database. Kaplan-Meier analysis was performed to show drug retention rates and Cox regression analysis was performed to investigate the factors affecting switching. Results Of the included 3138 SpA patients, 1165 (37.1%) switched to another biologic agent (switched group) and 1973 (62.9%) continued to receive their current therapies (continued group). The median follow-up duration of all patients was 3.8 years and the median time to switch was 1.0 years (0-13.4 years). According to the distribution of comorbidities, the rates of patients having diabetes mellitus, hyperlipidemia, asthma, gastrointestinal bleeding, and cancer were significantly higher in the switched group than those of in the continued group (8.4% vs. 5.8%, p=0.006; 14.5% vs. 9.2%, p In the switched group (n=1165), the main reasons for switching were secondary inefficacy (n=351), primary inefficacy (n=328), and side effects (n=267) followed by primary or secondary unknown inefficacy (n=57), physician’s request (n=45), patient’s demand (n=36), willing to be pregnant (n=9), other (n=37), and unknown (n=70). Conclusion In SpA patients, switching was frequent between anti-TNF agents and the median time to first switch was 1 year. Female gender, short disease duration, and lower BASDAI score were found to be the significant factors affecting switching from the anti-TNF agent used at first. The main reasons for this switching were primary (29.0%) and secondary (31.0%) inefficacy followed by side effects (23.6%). Switching between subcutaneous anti-TNF agents is generally less than switching from infliximab to another biologic agent. Disclosure of Interests Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Sedat Kiraz: None declared, Abdulsamet Erden: None declared, Orhan Kucuksahin: None declared, Timucin Kasifoglu: None declared, Ediz Dalkilic Grant/research support from: MSD and Abbvie, Consultant for: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Cemal Bes: None declared, Nilufer Alpay Kanitez: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, Abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, Abbvie,Celltrion, Novartis, Pamir Atagunduz: None declared, Belkis Nihan Coskun: None declared, Burcu Yagiz: None declared, Suleyman Serdar Koca: None declared, Muhammet Cinar: None declared, Askin Ates: None declared, Servet Akar Grant/research support from: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Speakers bureau: Pfizer, Onay Gercik: None declared, Duygu Ersozlu: None declared, Veli Yazisiz: None declared, Gezmis Kimyon: None declared, Muge Aydin: None declared, Ridvan Mercan: None declared, Burak Oz: None declared, Nazife Sule Yasar Bilge: None declared, Zeynel Abidin Akar: None declared, Omer Karadag: None declared, Ayse Bahar Kelesoglu Dincer: None declared, Sedat Yilmaz: None declared, Ufuk Ilgen: None declared, Yavuz Pehlivan: None declared, Ender Terzioglu: None declared, Levent Kilic: None declared, Sukran Erten: None declared, Ali Ihsan Ertenli: None declared

Neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), mean platelet volume (MPV), and red cell distribution width (RDW) may potentially reflect inflammatory status in systemic autoimmune diseases. The aim of this study is to investigate the association between these proposed markers and disease manifestations, activity, and severity in systemic sclerosis (SSc). We conducted a cross-sectional study of 69 systemic sclerosis (SSc) patients and 50 healthy volunteers in a single center. Adult patients with SSc and healthy controls were compared in terms of NLR, MLR, MPV, RDW, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). Venous blood samples were drawn after at least 8 h of fasting in the morning. Extension of skin fibrosis was evaluated by using modified Rodnan skin score (mRSS). Disease severity and activity were assessed by Medsger disease severity and European Scleroderma Trials and Research Group (EUSTAR) disease activity scores, respectively. Associations of disease manifestations, clinical, laboratory, and capillaroscopic findings, mRSS, and the disease activity and severity scores with the proposed hematological markers were evaluated. Multiple regression models were generated for significant associations. The neutrophil number was higher (p = 0.004) and lymphocyte number was lower (p

[No abstract available]

Liver is one of the most commonly involved organs in both primary and secondary systemic amyloidoses, but hepatic amyloidosis, manifested as mild to moderate enlargement, is usually not symptomatic nor it is clinically problematic. Rarely, massive hepatomegaly, severe cholestatic hepatitis or liver failure may be encountered in patients with systemic amyloidosis. Two cases with lambda light-chain amyloidosis presenting with nephrotic syndrome and atypical intrahepatic cholestasis are discussed with clinical features, laboratory and kidney, liver and bone marrow biopsy findings in view of the relevant literature.

Background/aim: The aim of this study is to determine the MEditerranean FeVer (MEFV) gene mutation carrier rate in patients with glomerulonephritis and to investigate the association between disease features and MEFV variants.Materials and methods: Medical records regarding clinical, laboratory, histopathological, and prognostic features of 200 adult patients with biopsy-proven glomerulonephritis were evaluated retrospectively. Exons 2 and 10 of the MEFV gene of each patient were sequenced by next-generation sequencing. Variants were detected and compared with disease features.Results: MEFV mutation carrier rate was 25%, similar among disease subgroups, and higher than the previously reported rates for normal populations. Demographic, clinical, and laboratory features at diagnosis did not differ in patients with and without mutations. Refractory disease rates were 73% and 40% in carriers and noncarriers of E148Q (P = 0.051).Percentage of global sclerotic glomeruli was higher in M694V carriers than noncarriers (medians 24% vs. 0%, P = 0.047). Tubulointerstitial fibrosis was also more severe in M694V carriers. The carrier rate of M694V was 14.3% in patients eventually needing chronic renal replacement therapy (RRT) (n = 21), whereas it was 2.8% in the group without RRT (OR = 5.8 [1.28-26.3], P = 0.040).Conclusion:MEFV mutation carrier rate was higher than expected in our sample of Turkish patients with glomerulonephritis. The E148Q mutation may be associated with refractory disease. The M694V mutation was more frequent in patients who needed chronic R RT.

Background/aim: We aimed to investigate the prevalence of anti-RNA polymerase (RNAP) III and other autoantibodies in a group of Turkish patients with systemic sclerosis (SSc) and their relation with clinical features. Materials and methods: The prevalence of anti-RNAP III and other autoantibodies was analyzed in 93 patients with SSc and control groups including 86 patients with systemic lupus erythematosus (SLE) and 65 healthy subjects, respectively. Their relationship with diseases findings was assessed in a cross-sectional manner. Results: Prevalences of anti-RNAP III were 2/93 (2.2%) in SSc, 1/86 (1.2%) in SLE, and 1/65 (1.5%) in the healthy group and there was no difference among groups (P > 0.999). Anti-Sm was significantly more common in SLE patients (P < 0.001), whereas antitopoisomerase I and anticentromere protein B were significantly more common in SSc patients (P < 0.001). There was a significant association between antitopoisomerase I positivity and interstitial lung disease (P < 0.001), and interestingly there was also a significant association between anti-SS-A 52 positivity and the presence of digital ulcers in patients with SSc. Conclusion: Our data show that anti-RNAP III in SSc patients was low in frequency in a Turkish population.

Objectives The aim of this study was to investigate the association between antiphospholipid antibodies and non-thrombotic and non-gestational manifestations of systemic lupus erythematosus. Methods Systemic lupus erythematosus patients with persistently positive antiphospholipid antibodies or lupus anticoagulant were identified and grouped as systemic lupus erythematosus with antiphospholipid syndrome (SLE-APS), systemic lupus erythematosus with positive antiphospholipid antibodies/lupus anticoagulant without antiphospholipid syndrome (SLE-aPL), and systemic lupus erythematosus with negative aPLs (SLE-No aPL). Groups were compared in terms of non-thrombotic systemic lupus erythematosus manifestations and laboratory features retrospectively. Results A total of 150 systemic lupus erythematosus patients, 26 with SLE-APS, 25 with SLE-aPL, and 99 with SLE-No aPL, were identified. Livedo reticularis, neurologic involvement, and thrombocytopenia were more common in antiphospholipid antibody positive systemic lupus erythematosus cases. Malar rash, arthritis, and pleuritis were more common in the SLE-No aPL, SLE-APS, and SLE-aPL groups, respectively. Positivity rates and titers of specific antiphospholipid antibodies did not differ between the SLE-APS and SLE-aPL groups. Conclusions Presence of antiphospholipid syndrome or persistent antiphospholipid antibodies may be related to non-thrombotic and non-gestational systemic lupus erythematosus manifestations. Patients with systemic lupus erythematosus plus antiphospholipid syndrome and persistent antiphospholipid antibodies without antiphospholipid syndrome also differ in terms of systemic lupus erythematosus manifestations.

Cytomegalovirus (CMV) infection and CMV disease, although quite rare in immunocompetent hosts, are associated with morbidity and mortality in immunocompromised patients. Because CMV infection is generally associated with high degree immunosuppression, beyond the cases with HIV infection and hematopoietic stem cell or solid organ transplantation, it is rarely seen in milder immunosuppressive states like in rheumatic diseases [1]. The exception is CMV colitis, prevalently seen in patients with inflammatory bowel disease (IBD) even in those not taking potent immunosuppressive agents [2]. Furthermore, there is limited data about upper gastrointestinal CMV disease in patients with rheumatic diseases taking immunosuppressive medication. Here, we describe severe CMV disease with esophagitis, pancytopenia, multiple large vein and catheter-related intracardiac thromboses along with central nervous system (CNS) findings in a case under immunosuppressive medication, previously diagnosed as pauci-immune glomerulonephritis.