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Objective: As long as immunosuppressive treatments are widely used, careful monitoring of HBVr is essential. We aimed to measure the awareness of Turkish physicians of certain specialties about the risk of hepatitis B virus reactivation (HBVr), HBV screening methods, prophylactic treatment practices and personal experiences in treating patients with biological agents. Methods: An electronic questionnaire link containing 21 questions about demographics and their applications to HBVr was sent to participants working in the various specialties across the country. Results: 227 participants took our survey (Female: 52.4%, n:119, Male: 47.6%, n:108), and consisted of 70 Hematologists, 65 Rheumatologists, 38 Gastroenterologists, 33 Oncologists, and 21 Dermatologists. We obtained extremely different results. It was found that all specialties are aware that the use of biological agents can cause HBVr (97.8%, n:222/227). Biological agents were used in 96% of the participants and HBVr was detected in 53.7% (Hematologist: 74.3%, Rheumatologist: 53.8%, Oncologist: 45.5%, Gastroenterologist: 44.7%, Dermatologist: 14.3%, p< 0.001). The general opinion was that, screening tests should be used (Gastroenterologist: 47.4%, p< 0.001) and 55.1% awared of how to monitor HBVr (Gastroenterologist: 65.8%, p = 0.06). The duration of antiviral prophylaxis was answered correctly in 70.4% (Hematologist: 82.6%, p = 0.01), the parameters of screening tests in 95.2% (Gastroenterologist: 97.4%, p = 0.52) and the highest risk of reactivation in 93% (Rheumatologist: 96.9%, p = 0.15) and the agent with the highest risk of reactivation in 82.8% of participants (Hematologist: 94.3%, p< 0.001). Conclusion: The opinions of different the clinical disciplines vary according to the profile of patients. Gastroenterologists' perspective on HBVr follow-up and treatment differs markedly from that of other clinical disciplines. However, to create general awareness and manage HBVr, guidelines for specific patient groups are definitely needed.

© 2023, The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).Background: This study aimed to assess the mortality of PsA before and during the COVID-19 pandemic. Methods: From the prospective, multicenter PsART-ID (Psoriatic Arthritis Registry-International Database), patients from Turkey were analyzed by linking the registry to the Turkish Cause of Death Registry. The outcome of interest was death from any cause, pre-pandemic (since the onset of registry—March 2014–March 2020), and during the pandemic (March 2020–May 2021). The crude mortality rate and standardized mortality ratio (SMR) were determined. Results: There were 1216 PsA patients with a follow-up of 7500 patient-years. Overall, 46 deaths (26 males) were observed. In the pre-pandemic period, SMR for PsA vs the general population was 0.95 (0.61–1.49), being higher in males [1.56 (0.92–2.63)] than females [0.62 (0.33–1.17)]. The crude mortality rate in PsA doubled during the pandemic (pre-pandemic crude mortality rate: 5.07 vs 10.76 during the pandemic) with a higher increase in females (2.9 vs 8.72) than males (9.07 vs 14.73). Conclusion: The mortality in PsA was found similar to the general population in the pre-pandemic era. The mortality rates in PsA doubled during the pandemic. Whether PsA patients have more risk of mortality than the general population due to COVID-19 needs further studies.Key Points• Decrease in mortality in PsA might be expected with the more effective treatment options and better disease control.• A crude mortality rate is comparable to the general population and not increased until the pandemic.• Currently, there is a 2-fold increase in crude mortality rate possibly due to the COVID-19.

The respiratory system is often affected by systemic sclerosis (SSc), a connective tissue disease characterized by fibrosis, vasculopathy, and inflammation. As a result, especially exertional dyspnea may occur in SSc patients. Evaluation of attitudes towards dyspnea is important in terms of preventing negative consequences such as kinesiophobia. However, no validated tool is available for assessing dyspnea-related kinesiophobia for patients with SSc. The aim of the present study was to perform the Turkish validation of the Breathlessness Beliefs Questionnaire for SSc (BBQ-SSc), which was adapted from the Tampa Kinesiophobia Scale, and to investigate its relationships with physical and clinical characteristics.Fifty patients with SSc (47 females) were included in the study. The patients were evaluated regarding pulmonary function tests, respiratory muscle strength, patient-reported dyspnea, quality of life related to respiratory problems, mood, and fatigue. The patients were re-evaluated 1 week later for the test-retest reliability of the BBQ-SSc.Internal consistency (Cronbach's alpha: 0.862) and test-retest validity (ICC: 0.831; 95% CI: 0.702-0.907) of the 12-item Turkish BBQ-SSc were found to be good. Principal component analysis confirmed the two-dimensional structure of the questionnaire. The scores of the questionnaire were associated with duration of illness, patient-reported dyspnea, quality of life related to respiratory problems, mood, and fatigue (p 0.05).According to our results, the 12-item Turkish BBQ-SSc is a reliable and valid tool to assess dyspnea-related kinesiophobia in SSc patients. Key Points • There are no structured tools available for assessing dyspnea-related kinesiophobia in patients with systemic sclerosis (SSc) • The 12-item Turkish Breathlessness Beliefs Questionnaire for SSc (BBQ-SSc) is a reliable and valid tool to assess dyspnea-related kinesiophobia in SSc patients.

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.

Using OCTA, investigate the capillary network and retinal layers in granulomatosis with ANCA associated vasculitis (AAV) patients who did not manifest apparent ocular involvement and compare the findings with healthy subjects.The present study, which is designed as a prospective and case-control study, includes 22 AAV patients and 35 control participants. OCTA parameters were noted.In most of the regions, AMT, RNFL and GC-IPL thicknesses were significantly lower in the AAV group than in the control group. While the vascular indices were lower in the AAV group, except for the center 1 mm region, the FAZ parameters were similar between the two groups.In AAV patients, subclinical changes in the retinal layers and superficial vascular plexus have been shown. In the future maybe a non-invasive method such as OCTA will become available in scoring systems for prognosis determination in AAV.

Objectives No studies examined the lower extremity–related anaerobic exercise capacity or functional status in adult patients with FMF. Methods Twenty-four patients with FMF (12 males) and 24 age–sex-matched healthy controls (13 males) were included in the study. Lower extremity–related anaerobic exercise capacity was assessed by using Wingate Anaerobic Test. Lower extremity–related functional status was examined by using 9-Step Stair Climb Test, 10-Repetition Chair Stand Test, and Six-Minute Walking Distance. Muscle strength of hip flexors, hip extensors, knee flexors, and knee extensors were evaluated by using a hand-held dynamometer. Results Patients with FMF had significantly poorer results in all anaerobic exercise capacity parameters and functional status assessments (P 0.05). Both average and peak anaerobic exercise capacities correlated significantly with all muscle strength measurements, 9-Step Stair Climb Test, 10-Repetition Chair Stand Test times, and Six-Minute Walk Distances (P Conclusion Lower extremity–related anaerobic exercise capacity and functional status seem to be diminished in adult patients with FMF. Evaluating these parameters may be beneficial for planning more appropriate and individualized treatment regimens such as patient education and exercise counselling for patients with FMF.

To investigate the effect of cervical stabilization exercises on cervical position error in patients with axial spondyloarthritis (axSpA).Thirty-nine patients with axSpA were randomly allocated to two groups as exercise group (n = 20, 11 males) and control group (n = 19, 12 males). The exercise group performed a progressive home-based cervical stabilization exercise program, while the control group did not receive any exercise intervention. To control exercise adherence and progression, text messages and video instructions were delivered via a freeware and cross-platform messaging service on a weekly basis. All patients were evaluated regarding physical characteristics, disease activity (Bath Ankylosing Spondylitis Disease Activity Index), functional status (Bath Ankylosing Spondylitis Functional Index), and spinal mobility (Bath Ankylosing Spondylitis Metrology Index). Cervical position error was evaluated in flexion, extension, rotation, and lateral flexion directions. All evaluations were performed at baseline and after 6 weeks.Baseline physical and disease-related characteristics were similar between the groups (p 0.05). After 6 weeks, significant improvements were observed in cervical position error in all directions in the exercise group (p 0.05), whereas no improvements were detected in the control group (p 0.05).A 6-week home-based cervical stabilization exercise program seems to be beneficial for improving impaired cervical proprioception in patients with axSpA.ZIEL: Ziel der Arbeit war es, die Wirkung von Übungen zur Stabilisierung der Halswirbelsäule auf deren zervikaler Positionsfehler bei Patienten mit axialer Spondyloarthritis (axSpA) zu untersuchen.Dazu wurden 39 Patienten mit axSpA randomisiert in 2 Gruppen eingeteilt, eine Übungsgruppe (n = 20, 11 m.) und eine Kontrollgruppe (n = 19, 12 m.). Die Übungsgruppe führte ein stufenweise aufbauendes Heimprogramm zur Halswirbelsäulenstabilisierung durch, während die Kontrollgruppe keine Übungsintervention erhielt. Um die Einhaltung der Übungen und ihren Fortschritt zu kontrollieren, wurden wöchentlich Textnachrichten und Videoanleitungen über einen freien und plattformübergreifenden Messagerdienst gesendet. Sämtliche Patienten wurden hinsichtlich körperlicher Merkmale, Krankheitsaktivität (Bath Ankylosing Spondylitis Disease Activity Index), funktionellem Status (Bath Ankylosing Spondylitis Functional Index), und Wirbelsäulenbeweglichkeit (Bath Ankylosing Spondylitis Metrology Index) untersucht. Zervikale Positionsfehler der Halswirbelsäule wurden in Flexion, Extension, Rotation und Lateralflexion beurteilt. Alle Untersuchungen wurden zu Anfang der Studie und nach 6 Wochen durchgeführt.Die körperlichen und krankheitsbezogenen Merkmale zu Anfang waren zwischen den beiden Gruppen ähnlich (p 0,05). Nach 6 Wochen wurden in der Übungsgruppe signifikante Verbesserungen der zervikalen Positionsfehler der Halswirbelsäule in allen Richtungen festgestellt (p 0,05), während in der Kontrollgruppe keine Verbesserungen zu beobachten waren (p 0,05).Ein 6‑wöchiges häusliches Übungsprogramm zur Stabilisierung der Halswirbelsäule scheint vorteilhaft zu sein, um die beeinträchtigte zervikale Propriozeption bei Patienten mit axSpA zu verbessern.

Background/aim: This study aimed to investigate the prevalence of sicca symptoms and secondary Sjogren's syndrome (SjS) in patients with systemic sclerosis (SSc). Also this study aimed to evaluate the expression of alpha-smooth muscle actin (alpha-SMA) in minor salivary gland (MSG) specimens, a possible marker of fibrosis responsible for myofibroblastic transformation. Materials and methods: Patients with SSc who were followed in Rheumatology outpatient clinic at a university hospital evaluated. The questionnaire of sicca symptoms and classification of SjS were evaluated according to the American-European Consensus Group (AECG) criteria. Histopathologic evaluations were done in MSG specimens investigating the presence of focal lymphocytic sialadenitis and glandular fibrosis, also assessing the expression of alpha-SMA. Results: This cross-sectional study included 102 patients with SSc [91 females (89%), mean age 52.5 +/- 12 years]. In this cohort 76 (75%) patients had sicca symptoms and 36 (35.3%) patients fulfilled the AECG criteria for SjS; all with limited form. Having SjS found to be associated with older age and the presence of positive anti-SS-A antibodies. On histopathologic examinations, glandular fibrosis was observed in 67 (80%) and lymphocytic sialadenitis was detected in 38 (45%) patients; but only 7 samples were positive for alpha-SMA. Conclusion: This study suggested sicca symptoms were found to be very common among patients with SSc. Also secondary SjS was detected in nearly one-third of patients with SSc; especially in limited subtype. Anti SS-A positivity and older age were detected as predictors for SjS. Histopathologic evaluations showed significant glandular fibrosis but rare alpha-SMA staining in patients with SSc.

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

Systemic sclerosis (SSc) is a connective tissue disorder characterized by collagen deposits in various organs. Skin involvement is one of the most common symptoms and along with vascular damage, may deteriorate hand functions. However, the status of hand functions has generally been investigated using patient-reported measures in patients with SSc. The aim of the present study was to investigate performance-based hand functions in patients with SSc using the Sollerman Hand Function Test (SHFT).A total of 39 patients with SSc (33 females) were included in the study. Twenty-four patients were classified as limited cutaneous SSc (lcSSc), while 15 patients were classified as diffuse cutaneous SSc (dcSSc). Hand-related physical characteristics were evaluated using the Modified Hand Mobility in Scleroderma Test, grip strength, and pinch strengths. The Duruoz Hand Index (Cochin Hand Functional Disability Scale), Disability of Arm, Shoulder, and Hand Questionnaire, Health Assessment Questionnaire, and Scleroderma Health Assessment Questionnaire were used as patient-reported measures. Performance-based hand functions were evaluated using SHFT.No significant differences were observed between lcSSc and dcSSc subtypes regarding performance-based and patient-reported hand functions (p 0.05). SHFT scores significantly correlated with hand-related physical characteristics and patient-reported hand functions (p 0.05). The highest correlation was determined between SHFT and the Duruoz Hand Index (rho: -0.652, p 0.001).According to our results, performance-based hand functions seem not to be affected by disease subtype. Performance-based hand functions may partially be captured by the patient-reported outcomes, especially the Duruoz Hand Index, in patients with SSc.ZIEL: Die systemische Sklerose (SSc) ist eine Bindegewebserkrankung, welche durch Kollagenablagerungen in verschiedenen Organen gekennzeichnet ist. Eines der häufigsten Symptome stellt die Hautbeteiligung dar, die in Kombination mit einer Gefäßschädigung auch die Funktionen der Hand beeinträchtigen kann. Allgemein wurde allerdings der Status der Handfunktionen bisher mittels Parametern gemäß den subjektiven Angaben von SSc-Patienten untersucht. Ziel der vorliegenden Studie war es, die leistungsbezogenen Handfunktionen bei Patienten mit SSc anhand des Sollerman Hand Function Test (SHFT) zu ermitteln.Dazu wurden 39 Patienten mit SSc (davon 33 Frauen) in die Studie einbezogen; bei 24 Patienten wurde die Erkrankung als limitierte kutane SSc (lcSSc) eingestuft, während sie bei 15 Patienten als diffuse kutane SSc (dcSSc) klassifiziert wurde. Handbezogene körperliche Merkmale wurden mithilfe des Modified Hand Mobility in Scleroderma Test, der Griffstärke und Kneifkraft beurteilt. Der Duruoz Hand Index (Cochin Hand Functional Disability Scale), Disability of Arm, Shoulder, and Hand Questionnaire, Health Assessment Questionnaire und der Scleroderma Health Assessment Questionnaire wurden als Parameter der subjektiven Angaben von Patienten eingesetzt. Die leistungsbasierten Handfunktionen wurden mittels SHFT beurteilt.Zwischen den Subtypen lcSSc und dcSSc wurden keine signifikanten Unterschiede in Bezug auf leistungsbasierte Handfunktionen und Handfunktionen nach patientenbezogenen Angaben (p 0,05) festgestellt. Die SHFT-Werte waren in signifikanter Weise mit den handbezogenen körperlichen Merkmalen und den Handfunktionen nach Patientenangaben korreliert (p 0,05). Dabei wurde die höchste Korrelation zwischen SHFT und dem Duruoz Hand Index (rho: −0,652; p 0,001) festgestellt.Den vorliegenden Ergebnissen zufolge scheinen die leistungsbasierten Handfunktionen nicht vom Krankheitssubtyp beeinflusst zu werden. Leistungsbasierte Handfunktionen bei Patienten mit SSc können mittels der von Patienten berichteten Ergebnisse erfasst werden, insbesondere mit dem Duruoz Hand Index.

Published by Elsevier Ltd., Background: Differences in the distribution of individual-level clinical risk factors across regions do not fully explain the observed global disparities in COVID-19 outcomes. We aimed to investigate the associations between environmental and societal factors and country-level variations in mortality attributed to COVID-19 among people with rheumatic disease globally. Methods: In this observational study, we derived individual-level data on adults (aged 18-99 years) with rheumatic disease and a confirmed status of their highest COVID-19 severity level from the COVID-19 Global Rheumatology Alliance (GRA) registry, collected between March 12, 2020, and Aug 27, 2021. Environmental and societal factors were obtained from publicly available sources. The primary endpoint was mortality attributed to COVID-19. We used a multivariable logistic regression to evaluate independent associations between environmental and societal factors and death, after controlling for individual-level risk factors. We used a series of nested mixed-effects models to establish whether environmental and societal factors sufficiently explained country-level variations in death. Findings: 14 044 patients from 23 countries were included in the analyses. 10 178 (72·5%) individuals were female and 3866 (27·5%) were male, with a mean age of 54·4 years (SD 15·6). Air pollution (odds ratio 1·10 per 10 μg/m3 [95% CI 1·01-1·17]; p=0·0105), proportion of the population aged 65 years or older (1·19 per 1% increase [1·10-1·30]; p, MAG is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K01 AR070585 and K24 AR074534 [JY]). KDW is supported by the Department of Veterans Affairs and the Rheumatology Research Foundation Scientist Development award. JAS is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K23 AR069688, R03 AR075886, L30 AR066953, P30 AR070253, and P30 AR072577), the Rheumatology Research Foundation (K Supplement Award and R Bridge Award), the Brigham Research Institute, and the R. Bruce and Joan M. Mickey Research Scholar Fund. NJP is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (T32-AR-007258). AD-G is supported by grants from the Centers for Disease Control and Prevention and the Rheumatology Research Foundation. RH was supported by the Justus-Liebig University Giessen Clinician Scientist Program in Biomedical Research to work on this registry. JY is supported by grants from the National Institutes of Health (K24 AR074534 and P30 AR070155).

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.

Abatacept is a fusion protein that blocks T cell activation. It is used in a variety of conditions including organ transplantation, immune deficiency, and autoimmune diseases. Even though abatacept-induced adverse events are observed, hypersensitivity reactions are rare. Desensitization protocols that can be implemented in the case of hypersensitivity reactions are present in the literature for many biological agents. However, a desensitization protocol for abatacept has not yet been established. Our patient, who was started on one of the biological agents, abatacept, for rheumatoid arthritis due to insufficient response to disease- modifying antirheumatic drugs, developed immediate hypersensitivity reaction with the first dose. Since it was planned to continue the treatment, abatacept desensitization was performed. The rapid desensitization protocol performed with prior premedication was successful and the patient was able to receive subsequent doses of abatacept using the same protocol. Keywords: Abatacept, anaphylaxis, desensitization

The study's aim was to investigate the relationship of grip strength and endurance with clinical characteristics, upper extremity functional disability, and quality of life in rheumatoid arthritis (RA) patients.We included 45 RA patients and 37 healthy controls in this cross-sectional study. All participants were evaluated using the maximal gripping test, static and dynamic grip endurance test, the Disability of Arm, Shoulder Hand Questionnaire, and Short Form-36, and sociodemographic and clinical characteristics were recorded.Moderate to strong correlations were found between functional disability and all of the grip-related variables. Physical, emotional, and social function sub-scores were correlated with grip strength on both sides and dynamic endurance on the dominant side.The study highlights grip-related variables associated with disability of the upper extremity. The grip strength was related to hand-wrist pain but not other clinical characteristics. Furthermore, grip strength on both sides and dynamic grip endurance on the dominant side were associated with some quality of life sub-scores. While therapists create a rehabilitation plan for RA patients, based on our findings, it may be beneficial to maximize therapeutic benefits on daily life and quality of life.

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.

Axial spondyloarthritis (axSpA) is a chronic inflammatory rheumatic condition, historically considered a predominantly male disease. However, increasing evidence suggests a more equal prevalence between men and women. Of the limited research conducted to date, it is apparent that gender differences exist in terms of time to diagnosis, treatment outcomes and health-related quality of life (HRQoL). Despite this, women are underrepresented in clinical trials and most studies do not stratify by gender to identify potential differences in terms of disease manifestations and treatment response. In this perspectives article, we reflect on the potential biological and social factors contributing to these differences and propose three key areas of education and research that should be prioritised in order to address the unmet needs of female patients with axSpA, namely: (1) to identify ways to increase awareness of disease occurrence in female patients among healthcare professionals (HCPs), (2) to improve understanding of gender differences in disease manifestation and outcomes, and (3) to conduct gender-stratified clinical trials with a representative sample of female patients.

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.

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.

Objective We aimed to evaluate quality of life (QoL), disease activity, compliance to treatment, patient and physician preferences for route of administration (RoA), status of health and pain in RA patients starting advanced treatments or needing a switch, and the factors associated with patient preferences. Methods A multicentre, prospective, observational and 1-year follow-up study was conducted, between 2015 and 2020, in adult RA patients using advanced treatments for the first time or needing a switch in their current treatments. All the data collected were entered into electronic case report forms. DAS in 28 joints with ESR [DAS28-4(ESR)], EuroQol 5-Dimensional Questionnaire (EQ-5D), HAQ Disability Index (HAQ-DI), Compliance Questionnaire for Rheumatology (CQR-19), Work Productivity and Activity Impairment Instrument (WPAI) and Patient Global Assessment-Visual Analogue Scale (PGA-VAS) questionnaires were used for longitudinal assessments. Results Four hundred and fifty-nine patients were enrolled. Three hundred and eight patients (67.1%) attended the final study visit at 12 months and were included for comparative analyses. Irrespective of RoA, the disease activity and QoL improved significantly at 12 months, whereas compliance worsened. At baseline and 12 months, EQ-5D and DAS28-4(ESR) scores were significantly correlated (P < 0.001). The WPAI scores changed significantly in favour of better outcomes over 12 months after initiation of advanced treatment or switching (P < 0.001). A higher proportion of patients preferred an oral RoA, in comparison to physicians (53.6% vs 31.4%; P < 0.001). Patient and physician RoA preferences were independent of gender, age, disease duration, advanced treatment type and the EQ-5D-3L, DAS28-4(ESR), HAQ-DI, PGA-VAS and CQR-19 scores at baseline. Conclusion The oral route was more frequently preferred by patients compared with physicians, although patients' preference rates showed a slight increase towards the end of the treatment, which might be an important factor for RA outcomes. Better control of disease activity and QoL were achieved at 12 months, regardless of RoA. Lay Summary What does this mean for patients? People with rheumatoid arthritis (RA) and their physicians can have different views throughout the patient journey, whether deciding the main treatment objective, switching a drug or deciding the route of drug administration. However, data are limited in this area. For this purpose, we have conducted a survey study to identify differences between the views of patients and physicians on the management of RA. In this study, we have shown that RA medication compliance decreases over time, irrespective of medication route. This is similar to other studies. We also spotted that there are different routes of drug adminstration (RoA) preferred: a higher proportion of patients preferred an oral RoA compared with physicians (53.6% vs 31.4%, respectively). Patient and physician RoA preferences were not related to gender, age, disease duration, treatment type and disease activity. By surveying patients and physicians at the same time, we have identified their differences better compared with previous studies. Patient preferences should have a major impact on disease management, and the results of this study might encourage patients to discuss their thoughts and preferences with their clinicians to achieve a better outcome., Pfizer; Pleksus CRO Inc., Pfizer has supported funding of this manuscript. Editorial/medical writing support was provided by Pleksus CRO Inc., and it was funded by Pfizer.

To evaluate the choroidal and retinal layers with optical coherence tomography (OCT) and retinal microvascular structures with optical coherence tomography angiography (OCTA) in systemic lupus erythematosus (SLE) patients. In this prospective, cross-sectional and comparative study, a total of 35 SLE patients and 35 healthy control participants were included. SLE patients who were using hydroxychloroquine (HCQ) and/or immunosuppressive agents are evaluated with OCT and OCTA. SLE patients who have no HCQ maculopathy observed in OCT were included in the patient group. Mean macular thickness and ganglion cell inner plexiform layer (GC-IPL) thicknesses were thinner in the patient group. When the parameters obtained with OCTA were evaluated, vessel (VD) and perfusion density (PD) were significantly lower in the patient group. Central foveal thickness and foveal avascular zone parameters were negatively correlated. In addition, VD and PD, and GC-IPL thicknesses were positively correlated. Application of OCTA for the evaluation of microvasculature in SLE patients may be useful in subclinical changes.

Objective Sjogren syndrome is a systemic inflammatory disease causing gland dysfunction. Few (and contradictory) reports on the mucosal effects of Sjogren syndrome have appeared. Here, we objectively demonstrate nasal dryness in Sjogren syndrome patients and explore the effect of such dryness on olfaction. Methods Thirty-four consecutive patients with primary Sjogren syndrome were enrolled in this cross-sectional study. The control group consisted of 21 age- and sex-matched volunteers. Medical histories and nasal findings were recorded. The Connecticut Chemosensory Clinical Research Center test was used to evaluate olfactory function. All subjects underwent mucucociliary clearance analysis (the saccharin test and peak nasal inspiratory flowmetry). The intranasal Schirmer test was used to evaluate the nasal cavity. Results The nasal Schirmer test scores were 8.4 mm (right) and 8 mm (left) (P = .041, P = .016, respectively, compared to controls). The Chi-squared test revealed significant differences (compared to controls) in nasal dryness (P = .001), postnasal drip (P = .04), and smell (a decrease) (P = .005). Neither olfactory function nor mucociliary clearance differed between the groups. We noted a trend toward a positive correlation between olfactory function and the nasal Schirmer score but statistical significance was not attained. Conclusion The intranasal Schirmer test objectively showed that Sjogren syndrome patients exhibited nasal cavity dryness; this is useful in terms of follow-up. This did not affect olfactory function. Level of evidence 4 Laryngoscope, 131:370-373, 2021.

The aim of this study is to evaluate the retinal and choroidal structures in r- and nr-axSpA patients using spectral domain optical coherence tomography (SD-OCT) and to compare changes with healthy controls. In this cross-sectional study, 70 axSpA patients (50 radiographic- and 20 nr-axSpA) and 50 healthy control subjects were included. Choroidal thickness (ChT), macular thickness, retinal nerve fiber layer (RNFL), and the ganglion cell complex (GCC) were measured by SD-OCT. For ChT values, seven lines at nasal and temporal were drawn at 500-μm intervals, centering the subfoveal sclerochoroidal junction. Analysis of the data was performed with the SPSS program. Mann–Whitney U test was performed for comparison of non-normally distributed continuous data; Student’s t test was used for normal distributed data. No significant difference was observed between 70 (66% male; mean age 39.7 ± 10.4 years) axSpA patients (50 radiographic and 20 nr-axSpA) and 50 (mean age 41.2 ± 6.2 years) healthy control subjects (p 0.417). R-axSpA and nr-axSpA groups and control group were similar in terms of spherical equivalent, intraocular pressure, axial length, and body mass index (p 0.574, p 0.874, p 0.918, p 0.344, respectively). While mean macular and GCC thicknesses were significantly lower in the patient group than in the healthy group, there was no significant difference between the two groups in terms of RNFL thickness. The present study showed that there was no significant relationship between markers and scores indicating disease activity and ChT, MT, RNFL, and GCC thicknesses. However, an increase in choroidal thickness and involvement of the retinal layers has also been demonstrated in patients with spondyloarthritis. In addition, the relationship between disease activity and retinal layer involvement is remarkable in the r-axSpA group.

OBJECTIVE: To evaluate whether there is any difference between radiographic axial spondyloarthritis (r-axSpA), also termed ankylosing spondylitis (AS), and non-radiographic (nr-) axSpA, with respect to subclinial myocardial dysfunction using speckle tracking echocardiography (STE). METHODS: This was a cross-sectional case control study. We included 72 patients with AS, 38 patients with nr-axSpA, and 56 age-matched healthy subjects. Patients with cardiac disease and cardiac risk factors affecting STE were excluded. The disease burden evaluated by the BASDAI, BASFI, BAS-G, and ASAS-HI scores were comparable in both the r- and nr-axSpA groups. A detailed echocardiographic examination including the M-mode, Doppler, and STE was applied to whole study population. RESULTS: Duration of the disease, the use of an anti-TNFα agent, and CRP levels were higher in patients with AS. Although the AS, nr-axSpA, and control groups had similar ejection fraction values (59±5.2, 60±4.6, 60±4.6, respectively, and p=0.499), the global longitudinal peak systolic strain (GLS) (20.5±3.3, 21.1±3.5, and 22.3±2.4, respectively, and p

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