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Background/Aim: Adult-onset Still’s disease (AOSD) is a multi-systemic, autoinflammatory disorder. Several activity scores have been proposed but none of them have been adopted universally. Our aim was to create a clinician-friendly activity scoring system by using simple clinical and laboratory parameters. Methods: AODS patients according to Yamaguchi criteria were included in this cross-sectional, multi-center study. Derivation and validation cohorts were constituted. Demographic, clinical and laboratory evaluation at study visit; patients’, and physicians’ global assessments of disease activity (both VAS/ Likert scale) were recorded. To develop the score; ordinal logistic regression model was used to determine independent predictors of patient’ global assessments of disease activity. Clinically and statistically significant variables were weighted according to regression coefficients. Then, performance of the score was tested on the validation cohort. Results: Total of 197 consecutive AOSD patient (125 in derivation, 72 in validation cohorts) were included. Final Still Activity Score was fever (2 points), arthralgia (2 points, plus 1 point if arthritis was present in ≥2 joints), neutrophilia≥65% (1 point) and ferritin≥350 ng/mL (1 point) (maximum of 7 points). The SAS yielded an AUC value of 0.98(0.96-1.00) in the derivation cohort and 0.91(95%CI:0.85-0.98) in the validation cohort to discriminate high AOSD activity from moderate-inactive AOSD. Correlation of SAS with PtGA was 83% for the derivation cohort and 76% for the validation cohort. Conclusions: SAS has shown a good test performance to distinguish active AOSD patients from the others. SAS may be a useful method for evaluating disease activity of AOSD patients in daily practice. Funding: None to declare. Declaration of Interest: None to declare. Ethical Approval: The present study was conducted in compliance with the Helsinki Declaration and was approved by the Local Ethics Committee of Hacettepe University (GO- 20/824). A written informed consent form was completed by all participants.

Background/aim: Familial Mediterranean Fever (FMF) is the prototype of hereditary autoinflammatory disorders and caused by mutations on the MEFV gene located on the short arm of chromosome 16. Although some MEFV variants are clearly associated with disease phenotype, there are numerous variants with unknown clinical association which are termed as variants of uncertain significance (VUS). Here, we present clinical correlations of VUS in a large cohort of adult FMF patients from three tertiary centers located in Central Anatolia. Materials and methods: All patients were recruited from FMF in Central Anatolia (FiCA) cohort. Demographic (sex, age at disease onset) and clinical features (disease characteristics, attack frequency, mean colchicine dose, colchicine nonresponsiveness, amyloidosis, and persistent inflammation) of patients with VUS were compared with those harboring pathogenic variants. Disease severity and damage were also evaluated using international severity score for FMF (ISSF) and autoinflammatory disease damage index (ADDI), respectively. Results: Among 971 participants included, MEFV gene analysis results were available for 814 patients. Twenty-six (3.2%) patients had single heterozygous VUS and 54 (6.6%) had pathogenic/VUS complex heterozygous variants. Patients with single heterozygous VUS had similar demographic/clinical features, ISSF and ADDI scores compared to those with single heterozygous pathogenic variant (p > 0.05 for all). No difference was observed in the demographic and clinical features of patients with single heterozygous pathogenic mutation and pathogenic/VUS complex heterozygous variant (p > 0.05 for all). ISSF and ADDI scores were lower in pathogenic/VUS complex heterozygous patients than those harboring single pathogenic mutation (p = 0.006 and 0.004, respectively). Conclusion: Our findings suggest that patients with single heterozygous VUS has mild FMF phenotype similar to those with single pathogenic mutation. Pathogenic/VUS complex heterozygosity does not lead to a more severe clinical phenotype than having a single pathogenic variant.

Background/aim: to evaluate treatment adherence and predictors of drug discontinuation among patients with inflammatory arthritis receiving bDMARDs within the first 100 days after the announcement of the COVID-19 pandemic. Materials and methods: a total of 1871 patients recorded in TReasure registry for whom advanced therapy was prescribed for rheumatoid arthritis (RA) or spondyloarthritis (SpA) within the 3 months (6-9 months for rituximab) before the declaration of COVID-19 pandemic were evaluated, and 1394 (74.5%) responded to the phone survey. Patients' data regarding demographic, clinical characteristics and disease activity before the pandemic were recorded. The patients were inquired about the diagnosis of COVID-19, the rate of continuation on bDMARDs, the reasons for treatment discontinuation, if any, and the current general disease activity (visual analog scale, [VAS]). Results: a total of 1394 patients (493 RA [47.3% on anti-TNF] patients and 901 SpA [90.0% on anti-TNF] patients) were included in the study. Overall, 2.8% of the patients had symptoms suggesting COVID-19, and 2 (0.15%) patients had PCR-confirmed COVID-19. Overall, 18.1% of all patients (13.8% of the RA and 20.5% of the SpA; p = 0.003) discontinued their bDMARDs. In the SpA group, the patients who discontinued bDMARDs were younger (40 [21-73] vs. 44 years [20-79]; p = 0.005) and had higher general disease activity; however, no difference was relevant for RA patients. Conclusion: although the COVID-19 was quite uncommon in the first 100 days of the pandemic, nearly one-fifth of the patients discontinued bDMARDs within this period. The long-term effects of the pandemic should be monitored., Hacettepe Rheumatology Society

Objective Behcet's disease is a complex systemic inflammatory vasculitis of incompletely understood etiology. This study was undertaken to investigate genetic associations with Behcet's disease in a diverse multiethnic population. Methods A total of 9,444 patients and controls from 7 different populations were included in this study. Genotyping was performed using an Infinium ImmunoArray-24 v.1.0 or v.2.0 BeadChip. Analysis of expression data from stimulated monocytes, and epigenetic and chromatin interaction analyses were performed. Results We identified 2 novel genetic susceptibility loci for Behcet's disease, including a risk locus in IFNGR1 (rs4896243) (odds ratio [OR] 1.25; P = 2.42 x 10(-9)) and within the intergenic region LNCAROD/DKK1 (rs1660760) (OR 0.78; P = 2.75 x 10(-8)). The risk variants in IFNGR1 significantly increased IFNGR1 messenger RNA expression in lipopolysaccharide-stimulated monocytes. In addition, our results replicated the association (P < 5 x 10(-8)) of 6 previously identified susceptibility loci in Behcet's disease: IL10, IL23R, IL12A-AS1, CCR3, ADO, and LACC1, reinforcing the notion that these loci are strong genetic factors in Behcet's disease shared across ancestries. We also identified >30 genetic susceptibility loci with a suggestive level of association (P < 5 x 10(-5)), which will require replication. Finally, functional annotation of genetic susceptibility loci in Behcet's disease revealed their possible regulatory roles and suggested potential causal genes and molecular mechanisms that could be further investigated. Conclusion We performed the largest genetic association study in Behcet's disease to date. Our findings reveal novel putative functional variants associated with the disease and replicate and extend the genetic associations in other loci across multiple ancestries., National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health (NIH) [R01AR070148]; NIH [U54GM104938, U19AI082714, UM1AI144292, P30AR053483, P30AR073750], Supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health (NIH) grant number R01AR070148 to Dr. Sawalha. Recruitment and genotyping of the EuropeanAmerican controls was supported by NIH grants number U54GM104938, U19AI082714, UM1AI144292, P30AR053483, and P30AR073750 to Drs. Guthridge and James. This work was supported by the use of study data downloaded from the dbGaP web site, under dbGaP accession phs000272. v1.p1.

Examination of antinuclear antibody (ANA) is used in diagnosis of systemic autoimmune diseases, and the indirect immunofluorescence (IIF) assay using HEp-2 cells is the gold standard method. HEp-2 allows the detection of multiple target antigen-directed autoantibodies. The guide “The International Consensus on ANA Patterns (ICAP)”, characterizes the patterns into three groups: nuclear, cytoplasmic, and mitotic. The majority of these are associated with autoimmune diseases, but some are rarely seen in autoimmune diseases or may be associated with conditions other than autoimmune disease. There is no consensus on how to report cytoplasmic and mitotic patterns-negative or positive. We aimed to examine the characteristics of patients that had cytoplasmic or mitotic staining in ANA evaluation by IIF. In our Medical Microbiology Laboratory, 18985 ANA tests of 16940 patients were studied between 01.01.2015-31.12.2019. Cytoplasmic or mitotic pattern was detected in 393 (2.07%) tests belonging to 385 patients. Cytoplasmic patterns suggestive of anti-mitochondrial antibody (AMA), anti-smooth muscle antibody (ASMA), anti-Jo-1 and anti-ribosomal P-protein were not included. The most common patterns were anti-midbody, anti-spindle fibers, and anti-vimentin patterns. There were 66 rheumatology patients that were negative for ANA but had cytoplasmic or mitotic staining. There was no statistically significant difference between the diagnosis and patterns of these patients. We suggest that the ANA should be reported as “negative” in case of cytoplasmic or mitotic pattern unless the term anti-cell antibody is used. It should be noted in the description part of the report in order to distinguish significant cytoplasmic patterns and give an idea for some specific conditions.

Background/Aims Neurologic involvement in Behcet’s syndrome (BS) remains an unelucidated area that needs further attention. In this study, we aimed to analyze the patterns of neurologic involvement and evaluate their association with other clinical features. Methods 800 patients with BS diagnosis according to The International Criteria for Behcet’s Disease (ICBD) followed in our center were retrospectively screened and 55 patients with central nervous system involvement and 20 patients with non-vascular BS were enrolled in this study. These patients were classified as sinus vein thrombosis (SVT), parenchymal involvement, and mixed (SVT and parenchymal). These groups were compared in terms of demographic and clinical features. Results Thirty-two (58,2%) of the patients were male. Distribution of neurologic involvement were as follows; 22 (40%) with isolated sinus vein thrombosis (SVT), 25 (%45) with isolated parenchymal, and 8 (%15) with mixed pattern. Central nervous involvement was the diagnostic feature of BS in 25 patients. Headache was the most common symptom followed by neurologic deficits and dizziness (44%, 25%, 16%). The most common sites of involvement were subcortical and periventricular areas followed by brainstem (63% vs 37%). In SVT group, positive family history of BS (p < 0.001) and previous deep vein thrombosis (DVT) (p:0.004) were found higher. Smoking history, erythema nodosum, cardiac and pulmonary involvement were significantly higher in parenchymal group (p < 0.001) (Table 1). Conclusion Although brainstem involvement is considered as more characteristic in BS, white matter and periventricular involvement were also found as common in our study. Notably, these subgroup analyses may suggest the speculation that BS shows clusters even within the neuro-vascular involvement. Disclosure R. Yıldırım: Other; Reşit Yıldırım is a member of Rheumatology journal as an associate editor. M. Dinler: None. A.E. Arslan: None. N.S.Y. Bilge: None. T. Kaşifoğlu: None.

Pulmonary involvement other than pulmonary artery involvement in Behcet's disease still remains an area of investigation. The aim of this study was to evaluate pulmonary involvement associated with Behcet's disease.We retrospectively investigated all Behcet's disease patients in terms of pulmonary involvement. Twenty-eight patients, whose radiologic examinations were consistent with Behcet's disease-related involvement after excluding other possibilities, were included in this study. Data regarding demographic characteristics, other clinical components of Behcet's disease, treatment modalities, and types of pulmonary involvement were analyzed.Pulmonary involvement was seen more common in male (82.1% vs 17.9%). Mean age for Behcet's disease diagnosis was found 32 years (SD 10.9) and mean age for pulmonary involvement was calculated 37 years (SD 11.4). Deep vein thrombosis (DVT) was the most common associated vascular involvement (53.6%). In our study population, alveolar hemorrhage and/or ground glass appearance were seen in 46.4% (13/28) of BD patients with pulmonary involvement. Totally, pulmonary artery aneurysm (PAA), small-sized pulmonary vasculitis (sPV), and pulmonary thrombosis (PT) were seen in 7 (25%), 13 (46.3%), and 18 (64.4%) of patients, respectively. Intracardiac thrombosis (ICT) in the right ventricle was present in 5 patients. Cyclophosphamide (CYC) was the most common preferred agent (78%) followed by azathioprine (AZA) in the first line. Warfarin was used in 18 patients. Overall mortality was seen in 3 patients: 1 due to PAA bleeding and others with unknown causes.Despite the importance of pulmonary artery involvement and pulmonary thrombosis in Behcet's disease, small-sized pulmonary vasculitis in the form of small vessel involvement is generally overlooked. Our study findings have shown that alveolar hemorrhage and/or ground-glass appearance in the absence of pulmonary artery aneurysm and pulmonary thrombosis are seen commonly as well. Key Points • The characteristics of pulmonary small vasculature involvement in Behcet's disease which is still an area of investigation warrant further attention. • The clinician should bear in mind that the spectrum of pulmonary involvement in Behcet's disease may be variable, but an extensive work up is still of great importance especially in atypical cases.

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.

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.

Objective Persistent inflammation is an insidious and less studied feature of FMF. We investigated clinical determinants of persistent inflammation and its associations with individual damage items. Methods This is a cross-sectional analysis of 917 FMF patients, who fulfilled the Tel Hashomer criteria and had at least 6 months’ follow-up. Patients were stratified based on whether they had persistent inflammation. We used logistic regression analysis to investigate independent predictors of persistent inflammation and the associated individual damage items. Results One hundred and forty-two (15%) patients had persistent inflammation. Active FMF (54%) was the most prominent reason for the persistent inflammation. Spondylarthritis (16%), other inflammatory arthritis (8%) and IBD (2%) were other frequent reasons. Male gender, history of exertional leg pain, inflammatory comorbidities, M694V homozygosity, colchicine resistance, lower education levels and musculoskeletal attack dominance were found to be the independent predictors of persistent inflammation. Earlier disease onset led to a tendency towards persistent inflammation. Patients with persistent inflammation were more likely to suffer damage. There is an increased risk of developing proteinuria, amyloidosis and renal insufficiency. Conclusion We identified, for the first time, the predictors of persistent inflammation in adult FMF patients and related individual damage items of the Autoinflammatory Disease Damage Index. Persistent inflammation is insidious and one of the chief causes of damage; therefore, especially patients with these predictors should be followed up more closely. If detected, underlying inflammatory comorbidities should be assessed meticulously as early detection and proper treatment strategies may favourably impact the natural history of the disease.

Background/aim Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in a substantial number of patients due to its side effects. In this study, we aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and cause permanent discontinuation. Materials and methods All patients were recruited from “FMF in Central Anatolia” (FiCA) cohort, 915 adults with a minimum follow- up time of 6 months during which they had obeyed all treatment instructions. Demographic and anthropometric data, FMF disease characteristics, disease severity, complications, and treatment features were recorded on a web-based registry. Prevalence of colchicine intolerance and characteristics of intolerant patients were analyzed. Results Effective colchicine doses cannot be maintained in 172 (18.7%) subjects. Main side effects that limit optimal dosing were as follows: diarrhea in 99 (10.8%), elevation in transaminases in 54 (5.9%), leukopenia in 10 (%1.1), renal impairment in 14 (1.3%), myopathy in five (0.5%), and allergic skin reaction in two. Colchicine had to be permanently ceased in 18 (2%) patients because of serious toxicity. Male sex and obesity were found to be associated with liver toxicity, and having a normal body weight was associated with diarrhea. Chronic inflammation and proteinuria were more common in colchicine-intolerant patients, and they had reported more frequent attacks compared to those tolerating optimal doses. Conclusion Colchicine intolerance is an important problem in daily clinical practice, mainly due to diarrhea and liver toxicity. Suboptimal colchicine dosing is associated with complications.

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration

OBJECTIVES: To analyse the clinical and laboratory factors associated with bamboo spine. METHODS: Data of patients fulfilling the 2009 ASAS classification criteria for axial spondyloarthritis, registered in the national, multicentre, longitudinal, and observational database of TReasure was analysed. Radiographs were assessed using the Bath Ankylosing Spondylitis Radiologic Index (BASRI). Data of patients with a bamboo spine (Group 1) was compared to data derived from patients with a longstanding disease of at least 15 years but no syndesmophytes (Group 2). RESULTS: Out of the 5060 patients, 1246 had eligible radiographs. There were 111 patients (8.9%) with a bamboo spine. Male sex was more common among patients with bamboo spine. The median BMI of 27.7 (25.8-31.1) in Group1 was higher than the BMI of 25.9 (22.9-29.2) in Group 2 (p

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.

[Objectives] Behçet's disease tends to be more severe in men than women. This study was undertaken to investigate sex-specific genetic effects in Behçet's disease., [Methods] A total of 1762 male and 1216 female patients with Behçet's disease from six diverse populations were studied, with the majority of patients of Turkish origin. Genotyping was performed using an Infinium ImmunoArray-24 BeadChip, or extracted from available genotyping data. Following imputation and extensive quality control measures, genome-wide association analysis was performed comparing male to female patients in the Turkish cohort, followed by a meta-analysis of significant results in all six populations. In addition, a weighted genetic risk score for Behçet's disease was calculated and compared between male and female patients., [Results] Genetic association analysis comparing male to female patients with Behçet's disease from Turkey revealed an association with male sex in HLA-B/MICA within the HLA region with a GWAS level of significance (rs2848712, OR = 1.46, P = 1.22 × 10−8). Meta-analysis of the effect in rs2848712 across six populations confirmed these results. Genetic risk score for Behçet's disease was significantly higher in male compared to female patients from Turkey. Higher genetic risk for Behçet's disease was observed in male patients in HLA-B/MICA (rs116799036, OR = 1.45, P = 1.95 × 10−8), HLA-C (rs12525170, OR = 1.46, P = 5.66 × 10−7), and KLRC4 (rs2617170, OR = 1.20, P = 0.019). In contrast, IFNGR1 (rs4896243, OR = 0.86, P = 0.011) was shown to confer higher genetic risk in female patients., [Conclusions] Male patients with Behçet's disease are characterized by higher genetic risk compared to female patients. This genetic difference, primarily derived from our Turkish cohort, is largely explained by risk within the HLA region. These data suggest that genetic factors might contribute to differences in disease presentation between men and women with Behçet's disease., This work was supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health grant number R01AR070148.

To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS).This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017.Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%).To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.

Objective Because of concerns about malignancy risks, using biological disease-modifying antirheumatic drugs (bDMARDs) in patients with a history of malignancy remains a challenging issue in rheumatology practice. This study aimed to investigate bDMARD preferences of physicians when treating of rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients with a history of malignancy. Methods The data for this cross-sectional study were gathered from the TReasure database using a date range of December 2017 and January 2020. Biological disease-modifying antirheumatic drug preferences were analyzed for 40 RA patients and 25 SpA patients with a history of malignancy. Results The most frequently prescribed bDMARD was rituximab, which was given to 28 RA patients (70%). For 25 patients (62.5%), the time between the diagnosis of malignancy and starting on a bDMARD regimen was less than 60 months, with a median interval of 43.5 months. Among SpA patients, the preferred bDMARDs were secukinumab and etanercept, which were each administered to 7 patients (28%). For 13 SpA patients (52%), the time between the diagnosis of malignancy and starting on bDMARDs was less than 60 months, with a median interval of 97 months. Conclusions The observed bDMARD preferences may be related to the therapeutic effects of rituximab on lymphoproliferative malignancies, the protective effects of secukinumab on tumor progression, and the short half-life of etanercept. Biological disease-modifying antirheumatic drugs should be used in RA and SpA patients with malignancy in case of high inflammatory activity.

To understand change in work productivity, activity impairment, quality of life (QoL), and disease activity in patients with psoriatic arthritis (PsA) receiving anti-tumor necrosis factor (anti-TNF) treatment.One hundred twenty patients with PsA receiving anti-TNF therapy were recruited to this noninterventional, observational study. Work disability was assessed via the Work Productivity and Activity Impairment (WPAI) questionnaire and disease activity was calculated via the 28-joint Disease Activity Score using C-reactive protein (DAS28-CRP) and Disease Activity Index for Psoriatic Arthritis with 28 joints (DAPSA28) score. Patient-reported outcomes (PROs), from visual analog scores and Health Assessment Questionnaire-Disability Index scores, were evaluated to understand the clinical effectiveness at baseline and every 3 months until the month-9 final visit. The American College of Rheumatology (ACR)20/50/70 response criteria were assessed at month 9.A total of 120 patients (females, n = 73) were enrolled in the study. Mean (SD) age and disease duration were 41.6 ± 11.1 years and 6.9 ± 6.5 years, respectively. The most commonly used TNFα inhibitor was adalimumab (42.4%), followed by etanercept (25.8%). All WPAI questionnaire parameters were reduced at the follow-up visits compared with baseline (p 0.001 for all). PROs and disease activity indicators (DAS28-CRP and DAPSA28) significantly improved during the course of anti-TNF treatments (p 0.001 for all). Additionally, ACR20/50/70 responses were determined as 86.8%, 63.7%, and 41.8% of patients at the month-9 visit.The real-world data in PsA patients receiving anti-TNF treatment showed improvement in WPAI, QoL, and disease activity over 9 months of treatment.NCT02028169 Key Points • Psoriatic arthritis (PsA), with debilitating effects on quality of life, occurs mostly in young adults and has negative impacts on employment status and work productivity. • Early PsA diagnosis and treat-to-target treatment strategies aim to reduce pain and joint damage, as well as improve work productivity. • Real-world data on the impact of treatment with anti-tumor necrosis factor (anti-TNF) agents on work productivity in PsA in the literature is scarce. • Our study of real-world data in patients with PsA receiving anti-TNF treatment showed improvement in work productivity, as well as in clinical and patient-reported outcomes.

We wanted to see how close we could get to our goal of treating rheumatoid arthritis (RA) without the use of glucocorticoids (GCs) in the disease-modifying antirheumatic drugs (DMARDs) era using real-life data. Established in 2017, the TReasure database is a web-based, prospective, observational cohort for Turkey. As of May 2019, there were 2,690 RA patients recorded as receiving biologic and targeted synthetic DMARDs (bDMARDs and tsDMARDs) therapy. At the start of the bDMARDs or tsDMARDs, patients with follow-up visits of at least 3 months were registered. At the time of registration and the last visit, doses of GCs were recorded and it was determined if the target dose of ≤ 7.5 mg was achieved. During registration and follow-up, 23.4% of the patients did not receive GCs and 76.5% of the patients received GCs at any time. GCs could be stopped after 59 (25–116) months in 28.4% of these patients, but 71.6% of patients were still using GC. The target GC dose could not be achieved in 18.2% of these patients (n = 352). The rate of continuing to use GC was significantly higher in women, in the elderly, those with rheumatoid factor (RF) positive, with higher Visual Analog Scale (VAS) pain and Disease Activity Score (DAS)-28. The initial GC dose of ≥ 7.5 mg/day was found to be crucial in not reaching the GC target dose (p

WOS: 000463722100007, PubMed ID: 30489254, 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.

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 (

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.

Background/aim: Peritonitis attacks of Familial Mediterranean Fever (FMF) usually requires emergency medical admissions and it's hard to distinguish a typical abdominal attack from surgical causes of acute abdomen. Therefore, history of abdominal surgery, particularly appendectomy, is very common in patients with FMF. However, history of appendectomy might also give some clues about the course of FMF in the adulthood. This study was to determine whether the history of appendectomy help to anticipate disease course of FMF in the adulthood. Materials and methods: All patients recruited from FMF in Central Anatolia (FiCA) cohort, comprising 971 adult subjects. All patients fulfilled the Tel Hashomer criteria. Demographic data, FMF disease characteristics, co-morbid conditions, past medical history, surgical history and disease complications were meticulously questioned and laboratory features and genotype data (if available) were recruited from patient files. Results: Appendectomy history was evident in 240 (24.7%) subjects. Disease onset was earlier and peritonitis is strikingly more prevalent (97.1% vs. 89.6%, p < 0.001) in appendectomized patients. These patients had reported almost two fold more frequent attacks in the last year compared to appendix intact patients (median 3.5 vs. 2 attacks, p = 0.001) without a difference in frequency of musculoskeletal and skin attacks. Severe disease was more common (10% vs. 5.9%, p = 0.038) due to involvement of more attack sites throughout the life and more frequent attacks. Appendectomy patients had used higher daily doses of colchicine to control disease (1.43 +/- 0.6 mg vs. 1.27 +/- 0.52 mg, p = 0.002) but colchicine resistance was also more common in these patients, 15% vs. 6.7% respectively, p < 0.001. Conclusion: Appendectomy history is common in FMF patients and associated with frequent serositis attacks in adulthood. These patients require higher colchicine doses with a lower rate of response and more need for Interleukin-1 antagonist therapies.

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.

The temporal relationship between the onset of skin psoriasis (Pso) and psoriatic arthritis (PsA) is a long and well-known feature of psoriatic disease. Psoriatic skin lesions emerge years before the development of musculoskeletal manifestations in nearly 75% of PsA patients. While skin and joint manifestations develop within one year in 15-20% of the patients, joint manifestations precede the skin involvement by a year or more in about 10% of the patients (1).

Behcet hastaligi, tekrarlayan oral ve genital ulserler, uveit ve tipik cilt lezyonlari ile bilinen sistemik bir vaskulittir. Notrofil-lenfosit orani (NLO) inflamasyon gostergesi olarak kullanilmasi arastirilan bir belirtectir ve ozellikle sub-klinik inflamasyon belirteci olarak kabul edilmektedir.Bu calismanin amaci vaskuler ve mukokutanoz tutulumu olan Behcet hastalarinda NLO’nun degerlendirilmesi ve saglikli kisilerden olusan kontrol grubu ile karsilastirilmasidir. Calismaya tromboz oykusu olan 20 Behcet hastasi (Grup 1), mukokutanoz tutulumu olan 20 Behcet hastasi (Grup 2) ve 20 saglikli gonullu (Grup 3) dahil edildi.Hastalarin dosyalari retrospektif incelendi, tam kan testindeki notrofil ve lenfosit degerleri kullanilarak NLO aritmetik olarak hesaplandi. Yas ve cinsiyet dagilimi gruplar arasi fark gostermemekteydi (p: 0,416 ve p:0,442, sirasiyla). Tam kan sayimi parametreleri degerlendirildiginde Grup 1’in hemoglobin degeri en dusuk, lokosit ve mutlak notrofil degerleri en yuksekti (p:0,045,p:0,033 ve p:0,003, sirasiyla). Grup 1’in NLO’ nun diger iki gruba gore yuksek oldugu, Grup 2 ve 3 arasinda fark olmadigi saptandi. (p:0,016; coklu analizde 1 vs 2; p:0,009, 1 vs 3; p:0,018,1 vs 3; p:0,807). Grup 1’de NLO ile hastalik aktivitesi ve siddeti arasindaki iliski incelendiginde korelasyon saptanmadi (aktivite icin: r:-0,212, p: 0,370 ve siddet icin r: -0,223, p: 0.344, sirasiyla). C-RP ile NLO korelasyonu sadece Grup 1’de saptanirken (r: 0,634, p: 0,03) hicbir grupta ESH ile NLO korelasyonu saptanmadi (p>0,05). Sonuc olarak,NLO’nun trombozu olan Behcet hastalarinda mukokutanoz tutulumlu olanlara veya saglikli kontrollere gore artmis oldugunu gosterdik. NLO, Behcet hastaligi gibi spesifik bir laboratuar gostergesi olmayan durumlarda artmis inflamasyonu veya hastalik aktivitesini degerlendirmede faydali olabilir.

Objective Familial Mediterranean fever (FMF), is an auto-inflammatory disease characterized by attacks of fever and serositis. Some scoring systems have been developed to evaluate the severity of the disease, however, predicting the severity of FMF is not possible with current knowledge. Our aim in this study was to evaluate the factors affecting disease severity in FMF. Methods This study included 150 FMF patients. The medical data of the patients were collected retrospectively and the International severity scoring system for Familial Mediterranean fever (ISSF) was used to evaluate disease severity. Results Patients were sorted into 3 groups based on the ISSF scores; ≤2=mild (Group 1; n: 61), 3-5=moderate (Group 2; n: 70), and ≥6=severe (Group 3; n: 19). Age at the onset of disease and age at diagnosis was younger in patients with severe disease (p: 0.009 and p: 0.031, respectively). Fever, peritonitis, and vasculitis were similar in all 3 groups. Pleuritis, erysipelas-like erythema (ELE), arthritis, myalgia, amyloidosis, and chronic kidney disease (CKD) were more common in Group 3. Conclusion FMF patients with early onset and early diagnosis, having more frequent pleuritis, ELE, arthritis, and myalgia tended to manifest a more severe form of the disease. Close monitoring of such patients may prevent the development of amyloidosis and CKD and improve the long-term prognosis of the disease.

Introduction/objectives Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis, but some patients may experience long-term complications of disease such as infertility/subfertility. The published data about FMF-associated infertility is still limited. The aim of this study is to investigate the frequency and to determine potential factors for FMF-associated infertility/subfertility. Methods We enrolled 971 adult patients with FMF. We defined infertility as the failure to conceive after 12 months of regular, unprotected intercourse. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics and genotype data (if available), disease complications, laboratory parameters, and treatment features were recorded. Results There were 582 subjects eligible for the present study (mean age 41.05 +/- 10.6 years, 65.8% female). MEFV mutations were available in 482 subjects, and 74.9% of subjects were harboring M694 V mutation (25.1% homozygous for M694 V). Infertility was present in 64 patients (14.6% of females and 4% of males). Multivariate analysis showed female sex [odds ratio (OR), 4.47; 95% confidence interval (CI95%) 1.75-11.42; p = 0.002], FMF disease onset < 20 years [OR, 2.99; (CI95% 1.04-8.61);p = 0.04], disease severity (ISSF) [OR, 4.81; (CI95% 2.28-10.17); p < 0.001], and colchicine nonresponse [OR, 2.80; (CI95% 1.17-6.74) p = 0.021] were the independent predictors of infertility. We also observed reversal of infertility in five patients who were treated with IL-1 beta antagonists. Conclusion Disease severity, FMF disease onset < 20 years, colchicine nonresponse, and female sex were found to be the independent predictors of infertility. The value of effective therapeutic interventions must be determined to treat infertility in these patients.center dot The prevalence of infertility increased in female patients with FMF.center dot Female sex, FMF disease onset < 20 years, disease severity, and colchicine nonresponse were risk factors for FMF-associated infertility.center dot With effective treatment of FMF, reversal of infertility was observed in five patients.

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet’s syndrome (BS). In this cross-sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28-year-old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement. © 2020 Japanese Dermatological Association

Tezcan, Mehmet Engin/0000-0002-1753-4936; TUFAN, Abdurrahman/0000-0001-6244-9362; SARI, Ismail/0000-0001-7737-4180; YILMAZ, Sedat/0000-0002-4691-3417 WOS: 000436403100017 PubMed: 28992387 ObjectiveApproximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. MethodsIn all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. ResultsIn total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. ConclusionAnti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Background:Suboptimal control of RA may lead to severe and progressive articular damage, loss of function, and deterioration of the quality of life (QoL).Objectives:To assess treatment satisfaction, sociodemographic, clinical, health care resource utilization, and QoL characteristics of patients with sub-optimally controlled RA and treated with conventional synthetic and/or biologic DMARDs.Methods:This study was an international, multicenter, cross-sectional, non-interventional study. Adult RA patients with moderate to severe disease activity (DAS28>3.2) were enrolled. Patient satisfaction was evaluated with Treatment Satisfaction Questionnaire for Medication (TSQM, version 1.4) with a scale ranging from 0 (indicating poor satisfaction) to 100 (indicating perfect satisfaction). Patients were questioned regarding treatment adherence, patient preferences, and expectations. Workability was evaluated using Work Productivity and Activity Impairment Questionnaire-Rheumatoid Arthritis (WPAI-RA, version 2.0). Short Form 36 (V2) survey were performed to all patients.Results:One hundred sixty-four patients were included in the study and most (78.0%) were female. The median age was 57.0 years, ranging between 22.0 and 84.0 years. Half of the patients (50.6%) were primary school graduates and 6.1% were unemployed due to RA and seeking work. Median time since RA diagnosis was 8.0 years and mean (±SD) DAS28-CRP score was 4.8 (±1.0). Mean total activity impairment was 54.9% (±27.4). In the past 3 months from enrollment, the mean number of healthcare professional and emergency room visits were 1.8 (±1.1) and 1.8 (±1.3), respectively. Mean number and length of hospitalizations in the previous 3 months were 1.1 (±0.3) times and 8.3 (±7.2) days, respectively. Mean TSQM scores were 53.5 (±21.4) for effectiveness, 86.0 (±26.7) for side effects, 67.8 (±16.5) for convenience, and 57.7 (±22.0) for global satisfaction. The leading expectation was ‘lasting relief of RA symptoms’ (mean score: 5.8). Preferred time until the effect of onset was ‘up to 1 week’ for 76.2% of the patients. Most of the patients (57.9%) preferred oral administrations and the most preferred frequency of administration was ‘once per day’ (46.3%). Mean physical and mental component summary scores for Short Form 36 (V2) survey were 37.9 (±8.3) and 40.1 (±10.7).Conclusion:Two-thirds of the patients with RA who have suboptimal responses are not satisfied with their treatments. Moreover, oral and once-daily treatment approaches stand out in patient preferences. Finally, suboptimal control leads to deterioration in clinical characteristics, workability, and QoL of patients with RA.Acknowledgements:The design, study conduct, and financial support for the study were provided by AbbVie. AbbVie participated in the interpretation of data, review, and approval of the publication. All authors have received research funding for this study. The authors wish to thank B. Murat Ozdemir of Monitor CRO for medical editing and reviewing services of this manuscript. AbbVie provided funding to Monitor CRO for this work.Disclosure of Interests:Umut Kalyoncu Speakers bureau: AbbVie, Pfizer, UCB, Novartis, and Janssen, Consultant of: AbbVie, Pfizer, UCB, Novartis, and Lilly, Grant/research support from: AbbVie, Pfizer, and Janssen, Adem Kucuk Speakers bureau: AbbVie, Gokhan Sargin: None declared, Fatih Ozdener Speakers bureau: UCB, Nutricia Advanced Medical Nutrition, Grant/research support from: Nutricia Advanced Medical Nutrition, Servet Yolbaş Speakers bureau: AbbVie, UCB, Pfizer, and MSD, Berna Yurttas: None declared, Sezin Turan: None declared, Gezmiş Kimyon Speakers bureau: AbbVie, Amgen, Pfizer, Novartis, UCB, MSD, Johnson and Johnson, and Celltrion, Consultant of: Amgen, and Pfizer, ALI SAHIN Speakers bureau: Roche, Pfizer, and AbbVie, Consultant of: Roche and Pfizer, Sedat Yilmaz Speakers bureau: UCB, Pfizer, AbbVie, MSD, Novartis, and Celltrion, Consultant of: Pfizer and Novartis, Ridvan Mercan Speakers bureau: AbbVie, Novartis, MSD, Pfizer, UCB, Roche, Amgen, and Celltrion, Consultant of: Novartis, MSD, Pfizer, and Celltrion, Hakan Emmungil Speakers bureau: AbbVie, Pfizer, Novartis, and MSD, Muhammet Çinar Speakers bureau: AbbVie, Pfizer, Celltrion, UCB, Amgen, Novartis, and MSD, Grant/research support from: AbbVie, Pfizer, Celltrion, UCB, Amgen, Novartis, and MSD, İlhan Sezer Speakers bureau: AbbVie, Pfizer, MSD, Novartis, Celltrion, UCB, Amgen, and Abdi Ibrahim, Consultant of: AbbVie, Pfizer, MSD, Novartis, Celltrion, UCB, Amgen, and Abdi Ibrahim, Grant/research support from: AbbVie, Pfizer, MSD, Novartis, Celltrion, UCB, Amgen, and Abdi Ibrahim, Timuçin Kaşifoğlu Speakers bureau: AbbVie, Amgen, Roche, MSD, Novartis, Pfizer, and UCB, Consultant of: AbbVie, Amgen, Roche, MSD, Novartis, Pfizer, and UCB, Fulya Cosan Speakers bureau: AbbVie, Pfizer, Novartis, UCB, and MSD, Taskin Senturk: None declared, Nevsun Inanc Speakers bureau: AbbVie, UCB, Novartis, Pfizer, Roche, Lilly and MSD, Consultant of: Roche and Pfizer, Grant/research support from: Roche and Pfizer

Objectives: Familial Mediterranean fever (FMF) is the most common hereditary auto-inflammatory disease. Our aim in the current study was to evaluate the relation between gender and clinical findings, the severity of disease and treatment compliance in FMF patients.Materials and Methods: Medical records of the 150 FMF patients were retrospectively evaluated. Disease severity scores were evaluated with the international severity score system for familial Mediterranean fever (ISSF), treatment compliance of the patients was evaluated with six-item modified Morisky scale and compared between males and females.Results: Of the 150 patients, 87 (58 %) were female and 63 (42 %) were male. Erysipelas like erythema (ELE) was more common in females (51.70% vs 30.90%, p= 0.03) and myalgia were more common in males (31.03% vs 36.50%, p=0.015). Other clinical findings were at similar frequencies.The ISSF scores of the groups were similar (2.93±2.05 vs 3.12±2.21, p= 0.716). The motivation for treatment and knowledge levels were the same in both groups (p=0.228 and p=0.112, respectively).Conclusion: Clinical findings, ISSF scores and treatment compliance of the FMF patients in both sexes are similar. The gender of the patient does not affect the clinical course of disease in FMF patients., Giriş: Ailevi Akdeniz ateşi (AAA) en sık görülen herediter, oto-inflamatuar hastalıktır. Bu çalışmada amacımız AAA hastalarında cinsiyet ve klinik bulgular, hastalık şiddeti ve tedavi uyumu arasındaki ilişkiyi incelemektir.Materyal ve Metot: AAA tanılı 150 hastanın arşiv dosyaları retrospektif olarak incelendi. Hastalık şiddeti uluslararası AAA şiddet skorlama sistemi (ISSF) ile, tedavi uyumları 6 maddeli modifiye Morisky ölçeği ile değerlendirildi ve cinsiyetler arası kıyaslandı. Bulgular: AAA tanılı 150 hastanın 87’si (%58) kadın ve 63’ü (%42) erkekti. Erezipel benzeri eritem (EBE) kadınlarda (%51,70 ve %19, p= 0,03) ve miyalji erkeklerde daha sıktı (% 31 ve % 36,50, p=0,015). Diğer klinik bulgular cinsiyetler arası farklılık göstermedi. ISSF skorları arasında da farklılık saptanmadı (2,93±2,05 ve 3,12±2,2; p=0,716). Tedavi uyumu ve bilgi düzeyleri her iki grupta benzerdi. (sırasıyla p=0,228 ve p=0,112,). Sonuç: AAA hastalarında klinik bulgular, ISSF skorları, tedavi uyumu cinsiyetler arası farklılık göstermemektedir. AAA hastalarında cinsiyetin prognoz üzerine etkisi yoktur.

Akutgeneralize ekzantematöz püstülozis (AGEP), genel olarak ilaç ilişkili birreaksiyon olup, ödemli eritem zemininde akut gelişen, folliküler olmayan, sterilpüstüller ile karakterize bir tablodur. Hidroksiklorokin(HCQ), antimalaryal bir ilaç olup romatizmal ve dermatolojik hastalıklarıntedavisinde yaygın olarak kullanılmaktadır ve AGEP’in nadir nedenlerindenbiridir. 44 yaşında kadın hasta romatoid artrit nedenli hidroksiklorokinalırken gelişen AGEP, HCQ’nin kesilmesi ve steroid tedavisinin başlanması ile düzeldi.Bu olgudaki amacımız romatoloji pratiğinde sık kullanılan bir ilaç olan HCQ kullanımısonrası gelişebilecek oldukça nadir istenmeyen bir durum olan AGEP ile ilişkilifarkındalığı arttırmak ve bu istenmeyen etkinin yönetimini belirlemek., Acute generalized exanthematous pustulosis (AGEP)is a clinical reaction pattern that is generally drug induced and characterizedby acute, nonfollicular sterile pustules on a background of edematous erythema.Hydroxychloroquine (HCQ) is anantimalarial drug which is widely used in dermatologic and rheumatologicdiseases and reported as a rare cause of AGEP. A44 years old female patient developed AGEP while recieving HCQ for rheumatoidarthritis and resolved after cessation of HCQ and starting steroid therapy. Theaim of this case is to raise the awareness about AGEP which is an uncommon andundesired effect of HCQ; a widely used drug in rheumatology practice.

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from "FMF in Central Anatolia (FiCA) Cohort", comprising 971 (mean age 35.3 +/- 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch-Schonlein purpura, Behcet's disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

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