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Background/aim The aim of this study was to evaluate the value of PET Vascular Activity Score (PETVAS) during the follow-up of patients with Takayasu arteritis. Materials and methods Takayasu arteritis patients who underwent 18F-Fluorodeoxyglucose (FDG) PET imaging were evaluated retrospectively. In 8 patients both 1 and 2-hour imagings were also performed prospectively. For PETVAS, 9 arterial areas were scored between 0-3 according to the FDG uptake. Results Forty-six images of 34 patients were evaluated. PETVAS was higher in patients with clinically active disease (p=0.03) and in C-reactive protein (CRP) elevated group among clinically inactive patients (p=0.0015). PETVAS correlated with CRP (p=0.003, r=0.53) and erythrocyte sedimentation rate (p=0.005, r=0.41), whereas age, disease duration, immunosuppressive and glucocorticoid (GC) treatments were not associated with PETVAS. First vs 2nd-hour PETVAS was similar in patients who had both 1st and 2nd hour PET scans (p=0.67). Conclusion We observed higher PETVAS in patients with active disease and elevated acute phase reactants. Although scores in our study (performed at one-hour) were lower compared to the original PETVAS study performed at two hours, PETVAS seems to be a reliable tool to quantify FDG PET scores in routine practice.

Objective: Metabolic syndrome (MetS) is one of the preventable risk factors for cardiovascular disease (CVD). The aim of this study was to investigate the effect of MetS on CVD and cumulative organ damage in a multi-center, large cohort of patients with Takayasu arteritis (TAK). Methods: This is a cross-sectional study involving 192 consecutive TAK patients from seven tertiary rheumatology centers in Turkey. Clinical data of TAK patients fulfilling the 1990 American College of Rheumatology classification criteria were collected from medical records. They were evaluated for risk factors of CVD, disease activity, damage, and MetS at their last visits. Results: A total of 192 consecutive TAK patients were included in this study. One hundred and fifty-eight (82%) were female, the mean age was 43.3 +/- 13 years, and mean disease duration was 13.5 +/- 9.3 years. MetS was detected in 50 (26%) of the patients and CVD was detected in 28 (14.6%). The presence of MetS was detected as an independent risk factor for CVD (P < 0.001). In addition, the mean vasculitis damage index of the group with MetS was significantly higher than in the other patients (4.5 +/- 3.3 vs 3.2 +/- 2.2, respectively, P = 0.004). Conclusion: The presence of MetS in TAK is associated with increased CVD and disease damage. Awareness and management of MetS can improve disease prognosis in patients with TAK.

Aim To compare childhood-onset (c-TAK) versus adult-onset Takayasu arteritis (a-TAK) patients for vascular involvement, disease activity, damage, and treatment. Methods Patient charts from two tertiary-care centers of a pediatric and adult rheumatology clinic were reviewed. Adult patients diagnosed before the age of 18 years were included in the childhood-onset group. The activity was assessed with the physician's global assessment (PGA) and Indian Takayasu Clinical Activity Score (ITAS). The damage was evaluated with Takayasu Arteritis Damage Score (TADS) and Vasculitis Damage Index (VDI). Results Twenty-four c-TAK (follow-up duration: 53 months) and 117 a-TAK patients (follow-up duration: 68 months) were analyzed. Aorta involvement was more prevalent (79% vs. 33%), and the median PGA score was higher in the c-TAK group (9 vs. 7), whereas the mean Indian Takayasu Arteritis Score was similar (14 vs. 13) among both groups. Median VDI score was lower for c-TAK patients (4 vs. 5), whereas TADS was similar for children and adults (8 vs. 8). Higher incidence of glucocorticoid related side-effects, a longer time to diagnosis and upper extremity claudication seemed to account for higher VDI scores in adults. Conclusion Aorta involvement was more common among children with TAK, whereas upper extremities were relatively spared. Biologic agents were used more commonly among children which may be explained by higher rates of aortic involvement. However, c-TAK patients did not have greater cumulative damage.

Takayasu arteritis is a rare inflammatory disease of large arteries. We performed a genetic study in Takayasu arteritis comprising 6,670 individuals (1,226 affected individuals) from five different populations. We discovered HLA risk factors and four non-HLA susceptibility loci in VPS8, SVEP1, CFL2, and chr13q21 and reinforced IL12B, PTK2B, and chr21q22 as robust susceptibility loci shared across ancestries. Functional analysis proposed plausible underlying disease mechanisms and pinpointed ETS2 as a potential causal gene for chr21q22 association. We also identified >60 candidate loci with suggestive association (p < 5 x 10(-s)) and devised a genetic risk score for Takayasu arteritis. Takayasu arteritis was compared to hundreds of other traits, revealing the closest genetic relatedness to inflammatory bowel disease. Epigenetic patterns within risk loci suggest roles for monocytes and B cells in Takayasu arteritis. This work enhances understanding of the genetic basis and pathophysiology of Takayasu arteritis and provides clues for potential new therapeutic targets., National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of HealthUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) [R01 AR070148]; National Institute of Arthritis and Musculoskeletal and Skin DiseasesUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) [U54 AR057319, U01 AR51874 04]; National Center for Research ResourcesUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Center for Research Resources (NCRR) [U54 RR019497]; Office of Rare Diseases Research of the National Center for Advancing Translational Sciences; Imperial College, National Institute for Health Research, Biomedical Research Centre; Wellcome TrustWellcome TrustEuropean Commission [WT107881]; Medical Research CouncilUK Research & Innovation (UKRI)Medical Research Council UK (MRC)European Commission [MC_UU_00002/4], This work was supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health grant R01 AR070148 to A.H.S. The Vasculitis Clinical Research Consortium has received support from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319 and U01 AR51874 04), the National Center for Research Resources (U54 RR019497), and the Office of Rare Diseases Research of the National Center for Advancing Translational Sciences. J.C.M., A.P.K., and R.M.M. acknowledge support from the Imperial College, National Institute for Health Research, Biomedical Research Centre. C.W. and G.R. acknowledge support from The Wellcome Trust (WT107881) and the Medical Research Council (MC_UU_00002/4). This work was supported by the use of study data downloaded from the dbGaP website, under dbGaP: phs000272.v1.p1, phs000431.v2.p1, phs000583.v1.p1, and phs000444.v1.p1.

Background This study aimed to examine fetomaternal outcomes in pregnant women in a large Turkish Takayasu arteritis (TAK) cohort and to evaluate the effects of pregnancy on the disease in those patients. Methods This is a cohort study involving 296 pregnancies of 112 TAK patients from 8 tertiary rheumatology centers in Turkey. Pregnancies were divided into 2 groups as pre-d (before disease onset) and post-d (after disease onset). In addition, post-d pregnancies were further divided into 2 subgroups according to fetomaternal complications (FMC) development status. Finally, patients were grouped into those with and without a history of pregnancy after disease onset. Results In post-d pregnancies, rates of worsening hypertension, new-onset hypertension, and preeclampsia were higher than in pre-d pregnancies (0.9% vs 16%, P < .001, 0.5% vs 5.3%, P = .012, and 0% vs 4%, P = .013, respectively). Patients with FMC were more likely to have renal artery involvement (65% vs 21%, P = .003). The patients who had post-d were younger, had longer disease duration, and had more relapses number than other patients (P < .001, P = .028, P = .016, respectively). Vasculitis Damage Index (VDI) results were similar in patients with or without post-d pregnancies. Conclusion Pregnancies after disease onset were found to be associated with HT and preeclampsia/eclampsia. HT-related FMCs are increased in TAK, and patients with renal artery involvement are at higher risk. The number of relapses increases in patients who become pregnant after disease onset, but pregnancy was not an independent risk factor for relapse. Pregnancy after the onset of disease had no negative effect on VDI.

Objectives Takayasu's arteritis (TAK) is a rare vasculitis characterized by inflammation of intermediate- to large-size arteries. Although pulmonary artery involvement (PAI) is an expected finding in some TAK patients, data on non-vascular pulmonary involvement (NVPI) are limited. We aimed to investigate the frequency of NVPI, including parenchymal infiltration, nodules/cavities, pleural effusion, and haemorrhage, in TAK. Method We assembled a retrospective cohort of TAK patients from nine tertiary centres in Turkey. The demographics and clinical characteristics of patients were extracted from medical records and the imaging findings were evaluated for pulmonary manifestations. Results As of January 2021, 319 TAK patients (female/male 276/43; mean age 42.4 +/- 13.5 years) were recruited. Eighty-two patients had cough and/or dyspnoea and four had haemoptysis as pulmonary symptoms. On computed tomography assessment, the overall frequency of NVPI was 7.2%; parenchymal infiltrations were present in 10 (3.1%), pleural effusion in eight (2.5%), nodules/cavities in six (1.9%), and pulmonary haemorrhage in four patients (1.3%). In the whole cohort, 10.3% of patients had pulmonary artery hypertension (PAH) and 5.6% had PAI. Among patients with PAH or PAI, the overall frequency of NVPI was significantly higher than in the rest of the group. Conclusions In this TAK cohort from Turkey, we observed NVPI in 7.2% of patients, with parenchymal infiltrations being the most common, followed by pleural effusion. Notably, NVPI was more frequent in patients with PAH or PAI. Although not as common as PAI, NVPI should be kept in mind, especially in TAK patients with PAH or PAI.

Objective: To compare the treatment outcomes of TNF inhibitors and tocilizumab (TCZ) in patients with Takayasu arteritis. Methods: Takayasu arteritis patients who were refractory to conventional immunosuppressive (IS) drugs and received biologic treatment were included in this multicenter retrospective cohort study. Clinical, laboratory and imaging data during follow-up were recorded. Remission, glucocorticoid (GC) sparing effect, drug survival was compared between TNF inhibitor and TCZ treatments. Also, a subgroup matched comparison was performed between groups. Results: One hundred and eleven (F/M: 98/13) patients were enrolled. A total of 173 biologic treatment courses (77 infliximab, 49 TCZ, 33 adalimumab, 9 certolizumab, 3 rituximab, 1 ustekinumab and 1 anakinra) were given. Tocilizumab was chosen in 23 patients and TNF inhibitors were chosen in 88 patients as first line biologic agent. Complete/partial remission rates between TCZ and TNF inhibitors were similar at 3rd month and at the end of the follow-up. GC dose decrease (

The aim of this study was to evaluate the relationships among the disease activity, illness perception, daily life performance, anxiety and depression status as potential mediators in Takayasu's arteritis (TAK) patients.In this cross-sectional study, 77 TAK patients were included. Data were collected by a clinical examination and a structured questionnaire regarding patient reported outcome measures (PROMs). Indian Takayasu's Arteritis Activity Score2010 (ITAS2010) was used to assess the disease activity (0: inactive vs ≥ 1: active). Illness Perception Questionnaire-Revise (IPQ-R), Work Productivity and Activity Impairment (WPAI) and Hospital Anxiety and Depression Scale (HADS) as PROMs were used to understand for the patient' perspective. After preliminary analysis, complex relationships among these variables were evaluated by mediation analyses in the study.WPAI-Daily impairment score, HADS-A and HADS-D scores as well as IPQ-R Consequence score were found be high in active TAK patients (p = 0.008; p = 0.001; p = 0.031; p = 0.001). HADS-D score was also correlated with WPAI-Daily impairment score and IPQ-R Consequence score (p0.05). In mediation analysis, IPQ-R Consequence score was directly mediated by disease activity (ITAS2010) (p = 0.0173) and indirectly mediated through HADS-D score (p = 0.0003). Similarly, HADS-D score was associated with poor WPAI-Daily impairment score in the mediation analysis in the indirect path (p = 0.0069). Disease activity (ITAS2010) also increased WPAI-Daily impairment score in direct path (p = 0.043).Active TAK patients perceived their illness more seriously and experienced more impairment in their daily life. Depression status as the mediator influenced them poorly. These interactions could give clues to improve PROMs in the clinical practice. Key Points •IIness perception, disease activity, mental status and daily life performance, assessed as patient-reported outcome measures, have a complex relationship in Takayasu's arteritis. •IPQ-R Consequence score, WPAI-Daily impairment score, HADS-Depression and HADS-Anxiety scores were found be high in active TAK patients. •In mediation analysis, IPQ-R Consequence score was directly mediated by disease activity and indirectly mediated through HADS-D score. Similarly, disease activity increased WPAI-Daily impairment score in direct and HADS-D in indirect paths.

Objectives. Certolizumab pegol (CZP) is a PEGylated antigen-binding fragment-fragment of a humanized mAb neutralizing TNF. It lacks Fc-fragment and has a very low potential to cross the placenta. We aimed to report the efficacy and safety of CZP in a case series of patients with refractory Takayasu arteritis (TA). Methods. Ten females of reproductive age (18-35 years) with TA were treated with CZP (at a dose of 400 mg at weeks 0, 2 and 4 and at 200 mg every 2 weeks thereafter) for a median of 10 months (range 3-28). Prior to CZP administration all patients received glucocorticoids and +/- MTX, CYC, AZA, HCQ, LEF or MMF. Six patients were previously treated with other biological anti-cytokine drugs. The National Institutes of Health criteria and the Indian Takayasu Clinical Activity Score 2010 were used to define disease activity. Results. All patients rapidly responded to treatment with CZP and were able to taper prednisone and MTX doses. Treatment with CZP resulted in a significant decrease in median serum CRP levels and normalization of Indian Takayasu Clinical Activity Score 2010 score in 9 of 10 patients. Remission of systemic vasculitis was achieved in all patients. Seven patients maintained remission for at least 4 months, while one patient developed relapse after 2 years of CZP treatment. Side effects included mild infections (n = 5). Conclusion. Our case series suggests that CZP may be an effective and steroid-sparing treatment option in patients with active TA even if they did not previously respond to other TNF inhibitors or tocilizumab.

Objective To assess the progression and the factors associated with damage in Takayasu‘s arteritis (TAK) patients during routine follow-up. Methods Patients diagnosed with TAK and had >6 months follow-up were enrolled in this study retrospectively. Takayasu's arteritis damage score (TADS) and vasculitis damage index (VDI) were determined at diagnosis and at the end of the follow-up and variables associated with damage scores were assessed. Results One-hundred fourteen patients (F/M: 101/13) were included in the study. The mean age at diagnosis, median symptom duration at baseline visit and mean follow-up duration were 35.3±13.3 years, 12 (0-360) months and 76.9±51.4 months, respectively. Median VDI score was 4.0 (1-8) and median TADS score was 7.0 (1-15) at baseline assessment. At the end of the follow-up, median VDI score increased to 5.0 (1-17) and TADS score to 8.0 (1-19). The median number of disease-related items were higher in TADS (8 items vs 4 items). At least one new corticosteroid (CS)-related damage item occurred in 35 patients (31%). Age at symptom-onset and cumulative CS doses were predictor factors for higher VDI score (≥5), whereas age at symptom-onset and disease duration were associated with increase in TADS (≥8). Gender and number of relapses were not associated with damage scores. Conclusion Damage assessment with VDI seems to capture treatment-related damage better, whereas TADS provides some additional information on disease-related damage in Takayasu's arteritis. Older age at symptom onset, disease duration and cumulative CS dose were associated with higher damage scores. The relapse frequency did not influence the damage level in our routine-follow-up of TAK patients.

Gastrointestinal (GI) system is commonly affected in sytemic sclerosis (SSc) patients who are also known to be at risk for malnutrition. We aimed to investigate the relationship between severity of GI disease, malnutrition and severity of organ involvement including microvasculopathy.A hundred and thirty-four SSc patients were included into the study; disease activity and severity, the University of California, Los Angeles, Scleroderma Clinical Trials Consortium Scleroderma Gastrointestinal scale 2.0 (UCLA SCTC GIT 2.0) and malnutrition universal screening tool (MUST) were cross-sectionally assessed. Nailfold video-capillaroscopy(NVC) was performed to evaluate microvasculopathy.SSc patients who are at medium to high risk for malnutrition (n=20); had more frequently limited pulmonary function, lung involvement, pulmonary hypertension, capillary rarefaction and NVC late pattern than those at low risk for malnutrition. Capillary rarefaction (≤6/mm) was shown to be independently associated with medium to high risk for malnutrition defined by using MUST. Capillary rarefaction and severe skin involvement were found to be independently related to 'severe' or 'very severe' GI disease defined by using UCLA SCTC GIT 2.0. UCLA SCTC GIT 2.0 scores were not found to be good discriminative in patients at risk for malnutrition allowing for a ROC curve of area under the curve (AUC)0.8).Assessment of gastrointestinal complaints and nutritional status by using symptom based questionnaires reflected the severity of GI disease and malnutrition including some limitations. Capillary rarefaction and severe skin involvement might be determining factors for malnutrition risk and severe GI disease.

Background: As a result of arterial ischemia, the frequencies of hypertension (HT), ischemic heart disease, congestive heart failure and atherosclerosis have been shown to increase and contribute to mortality in patients with Takayasu’s arteritis (TAK). Determining cardiovascular disease (CVD) and associated risk factors in TAK is important for a comprehensive treatment approach and better disease prognosis. Data about the effect of metabolic syndrome (MetS) which is known as a risk factor for CVD on TAK are limited. Objectives: The aim of this study was to determine the prevalence of MetS in patients with TAK and its effect on CVD and cumulative organ damage. Methods: A total of 122 TAK patients, followed by Turkish Takayasu Study Group in 7 tertiary Centers and diagnosed according to the 1990 ACR criteria were consecutively assessed for cumulative organ damage (VDI score), history of CVD and MetS as defined by the National Cholesterol Educational Program Adult Treatment Panel III (NCEP ATP III). CVD was defined as coronary artery disease or cerebrovascular event (myocardial infarction or stroke). Results: Eighty-seven percent of patients were female and the median age was 39 (17-65) years. The frequency of MetS was 14.7% and CVD was 13.1%. The median age, disease duration, smoking prevalence and CVD were found slightly higher in MetS group, without reaching statistical significance. There were no differences in VDI score between the groups (Table 1). Conclusion: MetS frequency in our TAK patients was observed to be less than the normal population data obtained from METSAR (Turkish MetS study in normal population) in Turkey (female: 39.6%, male: 28%). The discrepancy with SLE, which is another inflammatory autoimmune disease having a higher frequency of MetS related with organ damage, may be explained with the potentially more severe disease course in SLE patients requiring higher cumulative doses of glucocorticoids (1). Reference [1] Demir S, Artim-Esen B, Sahinkaya Y, Pehlivan O, Alpay-Kanitez N, Omma A, et al. Metabolic syndrome is not only a risk factor for cardiovascular diseases in systemic lupus erythematosus but is also associated with cumulative organ damage: a cross-sectional analysis of 311 patients. Lupus. 2016;25:177-84. Disclosure of Interests: Nilufer Alpay Kanitez: None declared, Sema Kaymaz Tahra: None declared, Ayten Yazici: None declared, Ayse Cefle: None declared, Mete Kara: None declared, Handan Yarkan-Tugsal: None declared, Onay Gercik: 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, Fatos Onen: None declared, Kenan Aksu: None declared, Gokhan Keser: None declared, Cemal Bes: None declared, Sevil Kamali: None declared, Fatma Alibaz-Oner: None declared, Haner Direskeneli: None declared

Background: Evaluation of damage in patients with takayasu arteritis is important because of the mortality and morbidity burden caused by the disease. Damage can be associated with treatment or with the disease itself (1). Objectives: In this study we aimed to evaluate the damage in our Takayasu arteritis patients by using VDI and TADS scores. Methods: Takayasu arteritis patients fullfilling the ACR 1990 criteria and had >3 months follow-up were enrolled in this study. TADS and VDI scores calculated at the end of the follow-up evaluated and compared. Results: 114 patients (F/M: 101/13) were included in the study. The mean age at diagnosis, median symptom duration at baseline visit and mean follow-up duration were 35.3±13.3 years, 12 (0-360) months and 76.9 ± 51.4 months respectively. Mean VDI score was 5.1±2.5 and mean TADS score was 7.9±3.5. At least one disease-related damage item was present in all of the patients for both VDI and TADS meanwhile ≥1 treatment-related damage item was established in 69 (66.1%) patients with VDI and 46(40.7%) patients with TADS scoring system. Median treatment related-item number was 1 (0-6) in VDI and 0(0-3) in TADS. This difference may be due to the lack of disease-related parameters such as diabetes, cataract, osteoporosis, avascular necrosis in TADS. The median number of disease-related items was higher in TADS scoring (4 items vs 8 items). TADS scores include more detailed and higher number of items under vascular intervention and pulse loss categories. Also bruit and systolic hypertension data is only available in TADS(Table1). There was no significant difference between patients with relapsing disease and patients with no relapses for both TADS and VDI scores. There was a weak corelation between VDI and cumulative steroid dose(p=0.002, r=0.32), and VDI-disease duration (p=0.001, r=0.29). A weak corelation between TADS and disease duration was established(p=0.04 r=0.19). No corelation was detected for TADS and cumulative steroid dose. Conclusion: In Takayasu arteritis, detecting the disease-related and treatment-related part of damage must be considered while monitoring the disease. VDI seems to be predominant evaluating the treatment-related damage. TADS provides more detailed information on disease-related damage. References [1] Omma A., Erer B., Karadag O.et al. (2017). Remarkable damage along with poor quality of life in Takayasu arteritis: cross-sectional results of a long-term followed-up multicentre cohort. Clin Exp Rheumatol., 35, 77-82. VDI: Vasculitis Damage Index TADS: Takayasu Arteritis Damage Score ENT: Eye-Nose-Throat ‘-‘ this item is not included in this scoring system ‘---‘ this item is categorized in another category in the scoring system mentioned Disclosure of Interests: None declared

Background Takayasu’ arteritis (TAK) is a rare vasculitis characterized by inflammation and obliteration of intermediate to large-size arteries. Even though more than 50% of patients with TAK have pulmonary artery involvement, non-vascular involvement and symptoms are uncommon. Objectives We aimed to investigate the frequency of non-vascular pulmonary involvement in TAK. Methods We assembled a retrospective cohort of patients with TAK from six different centers in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records, and the computed tomography findings were evaluated for pulmonary manifestations. Results As of January 2019, 197 TAK patients were recruited (mean age: 42.7±13.9 years [min-max: 17-75]) to the cohort, and 88.3% of them were female. Twenty-four patients had cough and/or dyspnea and four had hemoptysis as pulmonary symptoms. In CT assessment, parenchymal infiltrations were present in four (2%), pleural effusion in five (2.5%), nodule/cavity in one (0.5%), and pulmonary hemorrhage in one patient (0.5%). The patient who had pulmonary hemorrhage had also pleural effusion at the same time. In the whole cohort, 11.2% of patients (n=22) had pulmonary hypertension (PAH), three of them had cough and/or dyspnea and four of them had hemoptysis as a pulmonary symptom. Among patients with PAH, any pulmonary involvement in CT was more frequent compared to the rest of the patients (22.7% vs 5.1%, p Conclusion In this first assessment of Turkish TAK cohort, we observed non-vascular pulmonary involvement in about 5% of our patients and half of them were pleural effusions. The second most common manifestation was parenchymal infiltration with a frequency of 2%. Although rare, non-vascular pulmonary manifestations should also be investigated in TAK patients, especially in patients with pulmonary hypertension. References [1] Nakajima N. Takayasu Arteritis: Consideration of pulmonary involvement. Ann Vasc Dis2008;1(1):7-10. [2] Elsasser S, Soler M, Bollinger CT, et al. Takayasu disease with predominant pulmonary involvement. Respiration2000;67(2):213-5. Disclosure of Interests Ayten Yazici: None declared, Ayse Cefle: None declared, Sema Kaymaz Tahra: None declared, Nilufer Alpay Kanitez: None declared, Mete Kara: None declared, Onay Gercik: None declared, Handan Yarkan Tugsal: None declared, Fatos Onen: 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, Kenan Aksu: None declared, Gokhan Keser: None declared, Fatma Alibaz-Oner: None declared, Haner Direskeneli: None declared

Background Assessment of disease activity in systemic sclerosis (SSc) is challenging and usually hard to distinguish from damage or chronicity. Objectives We aimed to evaluate disease activity by different indexes and compare them in a SSc cohort. Methods Disease activity was evaluated by revised EUSTAR (European Scleroderma Trials and Research group) and EScSG (European Scleroderma Study Group) activity indexes in 131 SSc patients fullfiling ACR/EULAR classification criteria (2013). The patients with the scores of EUSTAR activity index ≥2.5 or EScSG activity index ≥3 were accepted as having active disease. Results Demographics, disease characteristics and nailfold video-capillaroscopic (NVC) pattern details were summarised in table-1. The scores of EUSTAR and EScSG activity indexes were correlated well (r=0.576, p=0.000) and the agreement between two scores for activity was moderate (cohen kappa:0.407). The percentages of SSc patients described as having active disease or not according to two activity indexes were summarised in table-2. Of the patients, 9.9% had active and 70.9% had inactive disease according to both indexes in this SSc cohort. Twenty -one (for EUSTAR) and 4 patients (for EScSG) were described as active according to one index and not to the other. Revised EUSTAR activity index was found to have 76.5% sensitivity and 81.6% specificity when the activity was defined by EScSG activity index. Conclusion This SSc cohort predominantly had limited cutanous disease, digital vasculopathy and late scleroderma pattern. Defining active disease was differed in 19% of the patients according to EUSTAR and EScSG activity indexes, former described higher frequency for activity. This difference might be related to validation procedures of these indexes in patients with different predominant stages of SSc disease, content of the index and the features of the cohort. Disclosure of Interests None declared

Giriş ve Amaç: Yeni oral antikoagülanların kullanıma girdiği bu ilk yıllarda herhangi bir antikoagülan ve/veya antiagregan kullanmakta iken gastrointestinal kanama sebebiyle hastanemize başvuran ve gastrointestinal sistem kanaması tanısıyla yatırılan hastalarda gastrointestinal sistem kanama sebepleri arasında yeni oral antikoagülanların yerini belirlemek ve ilaç grupları ile kanama ciddiyeti ve klinik tablo arasındaki ilişkilerin değerlendirilmesidir. Gereç ve Yöntem: Bu retrospektif çalışmaya antikoagülan ve/veya antiagregan kullanmakta iken gastrointestinal sistem kanama nedeniyle kliniğimize yatan toplam 178 hasta (ortalama yaş: 70±14 yaş) alındı. Hastalar yalnız antiagregan kullanan (n=124), yalnız antikoagülan kullanan (n=43) ve kombine ilaç kullanan (n=11) olarak üç ana gruba ayrıldı. Gruplar demografik veriler, biyokimyasal değerleri, 24 saatlik takip dilimlerinde maksimum hemoglobin düşüşleri, kan transfüzyonu miktarı ve mortalite oranlarına göre karşılaştırıldı. Bulgular: Hastaların ilaç kullanımına göre dağılımı; %70’inin antiagregan ilaç, %20’sinin varfarin, %4’ünün yeni oral antikoagülan, %6’sının kombine ilaç kullanımı şeklinde idi. Antiagregan ilaç grubunda %75 oranında aspirin kullanımı vardı. Antikoagülana bağlı kanamalarda ise varfarin %84, yeni oral antikoagülan ilaçlar %16 oranında tespit edildi. Antiagregan, antikoagülan ve kombine ilaç kullanım grupları arasında mortalite oranı (p=0.50), transfüzyon miktarı (p=0.72) ve maksimum hemoglobin düşüşleri (p=0.39) arasında fark saptanmadı. Yeni oral antikoagülanlar ile varfarin arasında morbidite ve mortalite oranlarında farklılık saptanmadı. Sonuç: Sonuç olarak yeni oral antikoagülanlar, antikoagülan ve/veya antiagregan kullanımı sonrası gastrointestinal kanama nedenleri arasında yer almaya başlamıştır. Varfarin ve yeni oral antikoagülanlarla ilişkili kanamaya bağlı morbidite ve mortalite oranları arasında anlamlı fark saptanmamıştır ancak bu ilaçların önümüzdeki yıllarda daha yaygın kullanımı ile gastrointestinal kanama etiyolojisindeki yerinin daha da artabileceği göz önüne alınmalıdır, Background and Aims: This study was designed to (i) address the position of new oral anticoagulants in the etiology of bleeding in patients admitted to our hospital for gastrointestinal bleeding while using any anticoagulant and/ or any antiaggregant drug and (ii) assess the relationships between the drug groups and severity of bleeding and the clinical picture in the first years of new oral anticoagulant use. Material and Methods: A total of 178 patients (mean age: 70±14 years) who were admitted to our clinic for gastrointestinal bleeding while using an anticoagulant and/or antiaggregant agent were recruited retrospectively. Patients were divided into the following three primary categories: patients using antiaggregant drugs (n=124), anticoagulant drugs (n=43), and both (n=11). The groups were compared according to their demographic data, biochemical parameters, 24-h follow-up period, maximum decrease in hemoglobin levels, amount of blood transfused, and mortality rates. Results: A total of 70% of the patients were taking antiaggregant drugs, 20% were using warfarin, 4% were taking new oral anticoagulants, and 6% of them were taking a combination of anticoagulants and antiaggregants. A total of 75% of the patients were using aspirin, 84% were taking warfarin, and 16% were taking new oral anticoagulants. There was no difference between the groups in terms of mortality rate (p=0.50), transfusion amount (p=0.72), and maximum hemoglobin decrease (p=0.39). There was also no difference in morbidity and mortality rates between patients taking new oral anticoagulants and those taking warfarin. Conclusion: Use of new oral anticoagulants has been listed as a cause for gastrointestinal bleeding. Although there was no difference between warfarin and new oral anticoagulant treatment with regard to mortality and morbidity rates, with the increasing use of these drugs in the forthcoming years, augmentation of their position in gastrointestinal bleeding etiology should be considered.