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Objectives To test the ability of an established traditional cardiovascular (CV) risk prediction score [Systematic COronary Risk Evaluation (SCORE)] and its EULAR modified version (mSCORE) to identify antisynthetase syndrome (ASyS) patients at high CV risk and to examine for the first time associations of CV and cerebrovascular surrogate markers with clinical and immunological ASyS parameters. Methods SCORE/mSCORE and the gold standard marker of aortic stiffness [carotid-femoral pulse wave velocity (cfPWV)] were examined in ASyS patients and healthy controls. Moreover, sonography of the common- (CCA) and internal- (ICA) carotid arteries was performed in subsets of both groups, evaluating carotid intima-media thickness (cIMT), plaques and Doppler sonographic cerebrovascular surrogates [resistance (RI) and pulsatility (PI) indices]. Results We recruited 66 ASyS patients and 88 controls. According to mSCORE, 10% of the patients had high CV risk. However, cfPWV and carotid sonography revealed an increased CV risk in 21.2% and subclinical carotid atherosclerosis (SCA) in 85.7% of the patients, respectively. cfPWV and cIMT were higher in patients compared with controls (Padj=0.021 and Padj=0.003, respectively). In the ASyS group, cfPWV and cIMT correlated significantly with age (r = 0.679; P Conclusion ASyS patients had higher aortic stiffness and SCA compared with controls, even after adjustment for confounders. SCORE/mSCORE performed poorly in identifying high-risk patients compared with cfPWV and carotid sonography. Thus, cfPWV and carotid sonography may improve CV and cerebrovascular screening in ASyS.

Objective.To examine the value of optical spectral transmission (OST) in detecting joint inflammation in patients with rheumatoid arthritis (RA) and to evaluate whether OST correlates with certain patient characteristics.Methods.OST measurements were performed in the metacarpophalangeal, proximal intraphalangeal, and wrist joints of 168 patients with RA and 114 controls. OST difference between the 2 groups was statistically examined and subsequently controlled for the effect of possible confounding factors. Diagnostic OST performance was tested by receiver-operating characteristics. Moreover, associations of OST with clinical and serological activity markers (patient group), joint ultrasound (US; patient subgroup) and various anthropometric and epidemiologic parameters (patient and control group) were evaluated by Spearman correlation coefficient and a generalized linear statistical adjustment model.Results.OST was significantly higher in the RA group than in the control group, even after adjustment for confounding factors (1.89; 95% CI 0.709–3.070, padj = 0.002). Taking US as a reference, area under the curve for all 1251 joints simultaneously was 0.67 (95% CI 0.631–0.709). In the patient group, correlation and adjustment analyses showed associations of OST with various disease activity markers [28-joint count Disease Activity Score (rho 0.313), swollen joint counts (rho 0.361), C-reactive protein (rho 0.389); all, padj = 0.001], age (rho 0.276, p < 0.001), and osteoarthritis (p = 0.022). Moreover, OST associated with a power Doppler US score (rho 0.442; p = 0.001) and a greyscale US score (rho 0.591; p < 0.001). In both groups males had significantly higher OST values than females and OST associated moderately weakly with body mass index (rho patients 0.316, rho controls 0.24; all, p < 0.001).Conclusion.Patients with RA showed higher OST values in comparison to controls. Moreover, OST associated with clinical, US, and laboratory disease activity markers.

© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Durch erhebliche versorgungsstrukturelle Defizite wird die Notwendigkeit einer moglichst fruhen Diagnose und Therapieeinleitung bei Rheumatoider Arthritis (RA) von der Mehrheit der Patienten in Deutschland verpasst. Die Herausforderung ist, den individuellen Anspruch eines bestmoglichen Therapieergebnisses mit einem gesellschaftlich tragfahigen Mittelaufwand in Einklang zu bringen. Das transsektorale Versorgungsnetzwerk ADAPTHERA zielt darauf ab, die fruhe RA-Diagnose und Behandlung in Rheinland-Pfalz zu verbessern. Fur die retrospektive Triage Analyse wurden 1045 Anfragen nachverfolgt. Zur Auswertung des klinischen Verlaufs wurde eine 143 Patienten umfassende Subkohorte analysiert. Quartalsweise wurden klinische und laborchemische Routineparameter erfasst, eine Batterie an Fragebogen ausgefullt und das therapeutische Regime bei Bedarf angepasst. Insgesamt konnten 454 Patienten eingeschlossen werden. Die mittlere Wartezeit betrug 23,9 (SD = 18) Tage. Der mittlere Beobachtungszeitraum in der Subkohorte lag bei 29,2 (SD = 12,7) Monaten, wobei die Halfte der Patienten binnen 3 Monaten vorstellig wurde. Fast 75 % der Patienten befanden sich nach 2 Jahren in Remission. Eine nachhaltige Remission konnte fur 74,8 % (6 Monate) respektive 53,5 % (12 Monate) beschrieben werden. Besonders Patienten mit rascher Remissionsinduktion profitierten im Sinne langerer Remissionen (p = 0,03). Ein sehr fruhes Krankheitsstadium (VERA) war mit einer seltener notwendigen Biologikatherapie assoziiert (p = 0,022). Der Ansatz eines Versorgungsnetzwerkes ist kein Allheilmittel, es kann aber eine deutliche Verbesserung der Versorgung bewirken. Kernpunkt zur Minimierung der kapazitaren Auslastung ist dabei eine moglichst zielgenaue Zuweisung und eine akkurate Triage.

BACKGROUND AND OBJECTIVE For patients with established rheumatoid arthritis and also early arthritis an increased prevalence of depression has been described. For a better understanding of depression in early arthritis patients, depression prevalences of a German early arthritis cohort were examined, with a focus on disease activity, anti-CCP status, disease duration and functional capacity over a period of 2 years. MATERIAL AND METHODS The evaluation was based on the early arthritis cohort ADAPTHERA from Rhineland-Palatinate. The inclusion criterion was a symptom duration before diagnosis of a maximum of 1 year. Data from the disease activity score 28 (DAS28), the Health Assessment Questionnaire (HAQ, functional status), the WHO-5 Well-Being Index (WHO-5, well-being and depressive symptoms) and the Patient Health Questionnaire-9 (PHQ-9, depressive symptoms) were collected. RESULTS At the beginning, 43.5% of patients had depressive symptoms (WHO-5 > 28). After the 2 year follow-up the percentage of patients with depressive symptoms had reduced to 20.8%. Correlations with disease activity according to DAS28 and the function of HAQ could be confirmed. There was no correlation between depressive symptoms and anti-CCP status (p = 0.431) or duration from symptom onset to diagnosis (p = 0.671). CONCLUSION Screening of early arthritis patients for the presence of depressive symptoms is of essential importance. Patients seem to be at high risk of developing depressive symptoms especially at the beginning of the disease and when showing high disability and poor results on disease activity score (DAS28 and visual analog scale).

Zusammenfassung Einleitung Ziel dieser Datenerhebung war die Untersuchung der klinischen, demografischen und Komorbidität-assoziierten Daten der Gicht sowie der klinischen Praxis der hausärztlichen Gichtversorgung in Rheinland-Pfalz (RL-P). Methoden Gicht-spezifische Fragebögen wurden über die kassenärztliche Vereinigung an alle Hausarztpraxen in RL-P versendet. Die Fragebögen setzten sich aus Items zur Erfassung von Epidemiologie, Medikation und Komorbiditäten zusammen. Darüber hinaus wurden Fragen zur klinischen Manifestation der Gicht mit einem besonderen Fokus auf therapierefraktäre Fälle eingebunden. Schließlich wurden die Hausärzte angehalten, den aktuellen Versorgungsstatus anhand einer Ratingskala zu bewerten. Ergebnisse Daten von 4016 Gichtpatienten (Durchschnittsalter zur Zeit der Diagnose: 62,8 Jahre, IQR 55 – 67,8) wurden anonym erhoben. Der überwiegende Anteil der Patienten war männlich (75 %) mit Podagra als häufigste Manifestationsform der Gicht (85 %). Chronisch tophöse Verläufe wurden in 15 % (Median 10 %, IQR 2 – 20) der Fälle angegeben und eine axiale Beteiligung in 2,7 % (Median 0 %, IQR 0 – 2). Durchschnittlich 11,3 % der Fälle (median 10 %, IQR 2,3 – 20) wurden als schwer behandelbar („hard-to-treat gout“) definiert. Trotz alledem wurden Biologika nicht namentlich als verwendete Behandlungsform erwähnt. 32 % der Patienten erhielten die Gichtdiagnose von ihrem Hausarzt, während 68 % weitere Ärzte aufsuchen mussten. Eine definitive Diagnose konnte nach durchschnittlich 3,1 Monaten (Median 0,3, IQR 0,1 – 1) gestellt werden. Diskussion Zusammenfassend gibt es im Zeitalter der Biologika einen Bedarf zur Optimierung der Gichtversorgung. Wichtige Ziele sind die Verkürzung der Diagnosezeit und die Prävention chronischer Verläufe. Die Schaffung eines gesteigerten Bewusstseins für die Erkrankung Gicht und ihre Komorbiditäten, ein standardisierter Behandlungsplan und Patiententraining könnten wichtige Schritte in diese Richtung sein.

To develop and validate a composite rheumatoid arthritis (RA) disease activity index using optical spectral transmission (OST) scores obtained with the HandScan, replacing tender and swollen joint counts.RA patients from a single center routinely undergoing HandScan measurements with at least 1 concurrent OST score and Disease Activity Score in 28 joints (DAS28) were included. Data were extracted from medical records. Linear regression analyses with the DAS28 as the outcome were performed to create a disease activity index (DAS-OST). OST score, erythrocyte sedimentation rate (ESR), and patient global assessment (PtGA) visual analog scale (VAS), sex, age, disease duration, and rheumatoid factor status were evaluated as independent variables. Final models were derived based on the statistical significance of coefficients and model fit. Of the data, two-thirds were used for development and one-third for validation; external validation was performed in a cohort from another center. Agreement between DAS-OST and DAS28 was assessed using the Bland-Altman plot method and intraclass correlation coefficient (ICC). Diagnostic value of the DAS-OST was determined for established definitions of remission, low disease activity (LDA), and high disease activity (HDA).Data of 3,358 observations from 1,505 unique RA patients were extracted. DAS-OST was defined as: -0.44 + OST × 0.03 + male × -0.11 + LN(ESR) × 0.77 + PtGA VAS × 0.03. The ICCs between DAS-OST and DAS28 were 0.88 (95% confidence interval [95% CI] 0.87-0.90) and 0.82 (95% CI 0.75-0.86) and measurement errors were 0.58 and 0.87 in internal and external validation, respectively. Sensitivity for remission, LDA, and HDA was 79%, 91%, and 43%, respectively, and specificity was 92%, 80%, and 96% in external validation.Using the HandScan, RA disease activity can be accurately estimated if combined with ESR, PtGA VAS, and sex into a disease activity index (DAS-OST).

Objectives To evaluate the influence of the SARS-CoV-2 pandemic on the adherence of patients with inflammatory rheumatic diseases (IRD) to their immunomodulatory medication during the three-month lockdown in Germany. Methods From 16th March until 15th June 2020, IRD patients from private practices and rheumatology departments were asked to answer a questionnaire addressing their behaviour with respect to their immunomodulating therapy. Eight private practices and nine rheumatology departments that included rheumatology primary care centres and university hospitals participated. A total of 4252 questionnaires were collected and evaluated. Results The majority of patients (54%) were diagnosed with RA, followed by psoriatic arthritis (14%), ankylosing spondylitis (10%), connective tissue diseases (12%) and vasculitides (6%). Most of the patients (84%) reported to continue their immunomodulatory therapy. Termination of therapy was reported by only 3% of the patients. The results were independent from the type of IRD, the respective immunomodulatory therapy and by whom the patients were treated (private practices vs rheumatology departments). Younger patients ( Conclusion The data show that most of the patients continued their therapy in spite of the pandemic. A significant change in behaviour with regard to their immunomodulatory therapy was not observed during the three months of observation. The results support the idea that the immediate release of recommendations of the German Society of Rheumatology were well received, supporting the well-established physician–patient relationship in times of a crisis.

The current COVID-19 pandemic inherits an unprecedented challenge for the treating rheumatologists. On the one hand, antirheumatic drugs can increase the risk of infection and potentially deteriorate the course of an infection. On the other hand, an active inflammatory rheumatic disease can also increase the risk for an infection. In the recommendations of the German Society for Rheumatology (www.dgrh.de), it is recommended that our patients continue the antirheumatic therapy to maintain remission or low state of activity despite the pandemic. In this study, patients with inflammatory rheumatic disease were asked in the first weeks of the pandemic on their opinion of their immunomodulating therapy. The result shows that over 90% of the patients followed the recommendation of the rheumatologist to continue the antirheumatic therapy, and only a small percentage of the patients terminated the therapy on their own. This result was independent of the individual anti-rheumatic therapy. Taken together, the results of this study illustrate not only the trustful patient-physician partnership in a threatening situation but also the high impact of state-of-the art recommendations by the respective scientific society.

Despite recent improvements in the treatment of systemic lupus erythematosus (SLE), disease activity, comorbidities and drug toxicity significantly contribute to the risk of progressive irreversible damage accrual and increased mortality in patients with this chronic disease. Moreover, even lupus patients in remission often report residual symptoms, such as fatigue, which have a considerable impact on their health-related quality of life. In recent decades, SLE treatment has moved from the use of hydroxychloroquine, systemic glucocorticosteroids and conventional immunosuppressive drugs to biologic agents, of which belimumab is the first and only biologic agent approved for the treatment for SLE to date. Novel therapies targeting interferons, cytokines and their receptors, intracellular signals, plasma cells, T lymphocytes and co-stimulatory molecules are being evaluated. In the context of a holistic approach, growing evidence is emerging of the importance of correct lifestyle habits in the management of lupus manifestations and comorbidities. The aim of this paper is to provide an overview of current pharmacological and non-pharmacological treatment options and emerging therapies in SLE.

Introduction The aim of this study was to determine the prevalence of methicillin-resistant Staphylococcus aureus (MRSA) both in rheumatologic and non-rheumatologic rehabilitation centers. In addition, we sought to evaluate the practice value of existing screening recommendations of the German Commission for Hospital Hygiene and Infection Prevention (KRINKO). Methods The analysis was performed in four rehabilitation clinics (rheumatology, psychosomatic medicine, oncology, and cardiology) with at least 200 patients per clinic tested for MRSA. Results Nine (1.1%) of the 842 patients were colonized with MRSA. Only five of them should have been tested according to the commission’s recommendations. The prevalence was 0.5% (n = 207) in rheumatologic, 0.9% (n = 224) in psychosomatic, 1.4% (n = 209) in oncologic and 1.5% (n = 202) in cardiologic patients. We found a greater exposure to risk factors in cardiologic and oncologic patients. Among patients with carrier status, a higher percentage was exposed to three potential risk factors not applied by the commission. Conclusions The prevalence of MRSA in our cohort correlates with data from previous studies. The low percentage among rheumatologic patients suggests that they are not more likely to reveal MRSA carrier status than other patient groups and that long-term immunosuppression does not necessarily represent a risk factor for MRSA colonization. Since only five out of nine patients with carrier status would have been detected following the recommendations of the KRINKO, further studies on potential risk factors are warranted.

Objective Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. Methods Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. Results 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9–51) and 40 (24%) developed new accompanying features after 19months median (IQR 6–56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68–9.21, p=0.002). Conclusion Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.

Background: Macrovascular involvement and cardiovascular (CV) risk have not been sufficiently studied in patients with mixed connective tissue disease (MCTD). In particular, the gold standard assessment method of aortic stiffness carotid-femoral pulse wave velocity (cfPWV) (1) has never been evaluated in patients with this disease. Objectives: Aims of the present study were to examine cfPWV in MCTD and to evaluate its associations with MCTD associated parameters and traditional CV risk factors. Methods: cfPWV measurements were performed in 43 MCTD patients and 107 healthy controls. The difference between cfPWV in the two groups was statistically examined and subsequently controlled for the effect of possible confounding factors. Association of cfPWV with MCTD associated organ involvement, routine laboratory parameters and immunoserological markers was also evaluated. Finally, relationship of cfPWV with medications and traditional CV risk factors was examined. Results: Adjusted statistical analyses for confounding factors showed significantly higher cfPWV values in MCTD patients in comparison to controls (padj Conclusion: To our knowledge, this is the first study to show that patients with MCTD have significantly higher aortic stiffness and thus CV risk in comparison to controls. Except the disease itself, age and blood pressure were the main predictors of cfPWV. References [1] Bortel LM Van, Laurent S, Boutouyrie P, et al. Expert consensus document on the measurement of aortic stiffness in daily practice using carotid-femoral pulse wave velocity. [2] J Hypertens2012; 30: 445–448. Disclosure of interests: Konstantinos Triantafyllias: None declared, Michele De Blasi : None declared, Freya Lutgendorf: None declared, Lorenzo Cavagna: None declared, Marco Stortz: None declared, Julia Weinmann-Menke : None declared, Stavros Konstantinides : None declared, Peter Galle : None declared, andreas Schwarting Grant/research support from: GSK, Pfizer, abbVie, Novartis, Roche, Speakers bureau: GSK, Novartis

Background Rheumatoid arthritis (RA), psoriasis arthritis (PsA) and spondyloarthritis (SpA) are the most common chronic autoimmune rheumatic diseases. For all 3, the so-called “window of opportunity” [1-3] has been identified as the decisive factor affecting the outcome. Objectives The aim of the prospective study is to improve early diagnosis of RA, PsA and SpA and thus positively impact the quality of care for patients with the help of coordinating centers. The primary endpoint is the number of cases with an actual indication for immediate referral to a specialist in proportion to all reported cases. Methods The project establishes a network of the University Medical Center Mainz, the Medical School Hannover, the Center for Rheumatology Lower Saxony e.V., the University Medical Center Saarland, the ACURA Center of Rheumatology Rhineland-Palatinate, the Leibniz University Hannover and the University Medical Center Freiburg, local rheumatology specialists, the associations of statutory health insurance physicians and general practitioners and the 3 regional associations of the Deutsche Rheuma-Liga e.V. Covering 3 federal states, the network reaches approximately 13 million inhabitants. Primary care providers are given access to screening questionnaires to document potential early cases of RA, PsA, and SpA, based on characteristic symptoms. These are evaluated by multidisciplinary teams at the regional coordinating centers. If they fulfill the criteria for referral, patients get an appointment at a cooperating rheumatology specialist within weeks. If a rheumatic disease is diagnosed, physicians and patients receive questionnaires on sociodemographic data, quality of life, functionality, medication, diagnosis and psychological aspects. After 12 months, the questionnaires are completed a second time. The collected data is compared with a matched reference group of the German Rheumatism Research Center. It is planned to screen up to 8.700 people within the 30-month survey phase that started in October 2017. Results Preliminary data will be presented at the conference. Currently, 2.400 patients have been screened, of which 400 patients were included in the study and 15 already underwent their follow-up screening. Conclusion Previous studies have shown positive results of coordinated cooperation among the relevant care providers. For example, 75 percent of the patients diagnosed with early RA within the ADAPTHERA network were in remission after 2 years (DAS28 References [1] O’Dell, J.R., Treating rheumatoid arthritis early: a window of opportunity? Arthritis Rheum, 2002. 46(2): p. 283-5. [2] Boehncke, W.H. and A. Menter, Burden of disease: psoriasis and psoriatic arthritis. Am J Clin Dermatol, 2013. 14(5): p. 377-88. [3] Claudepierre, P., Spondyloarthritis: a window of opportunity? Joint Bone Spine, 2014. 81(3): p. 197-9. [4] Lauter, A., et al. ADAPTHERA - Landesweit transsektorales Versorgungsnetzwerk fur Patienten mit fruher rheumatoider Arthritis zeigt anhaltende Remissionen in der Regelversorgung. Wunder Punkt: Die Triage. (2018). Manuscript. Disclosure of Interests Matthias Dreher: None declared, Gunter Assmann: None declared, Kirsten Hoeper: None declared, Konstantinos Triantafyllias: None declared, Jan Zeidler: None declared, Harald Binder: None declared, Reinhold E. Schmitt: None declared, Andreas Schwarting Grant/research support from: GSK, Pfizer, AbbVie, Novartis, Roche, Speakers bureau: GSK, Novartis

Background Early diagnosis is crucial for patients with rheumatic diseases. Studies show that early stage patients already show reduced mental and physical health [2, 3]. However, early diagnosis is often delayed due to the severe lack of rheumatologists in Germany. Objectives The cross-sectional study compares data on physical and mental health of known and suspected early rheumatic patients, collected from participants of the bus tour. Methods The Rheuma-Bus-Tour is an annual, two week open-access screening event in three states (Rhineland-Palatine, Saarland, Lower Saxony) that raises awareness for rheumatic diseases and identifies people with potential early cases of RA, SpA and PsA. All participants, regardless of their diagnosis status, completed a screening questionnaire about early symptoms, sociodemographic as well as physical and mental health parameters: Habitual Physical Activity Questionnaire (HPA) [1], Hannover Functional Ability Questionnaire (FFbh-R), Patient Health Questionnaire (PHQ-9), Well-Being Index (WHO-5). Additionally, a CRP on-site test and a medical consultation were offered. Results In total, 853 participants completed the questionnaire, 214 with a known rheumatic diagnosis and 626 without one. Of 533 conducted CRP tests, 107 were positive. After the consultation, 58 patients were referred for an immediate rheumatologist appointment through the Rheuma-VOR network, 16 of which were diagnosed with RA, SpA, or PsA. Tables 1 and 2 present the group differences between the already diagnosed and the not diagnosed groups, split by CRP results. * Significant at p ≤ 0.05. Average age for all three groups falls in the 50-59 year range. Differences were found between PHQ-9, WHO-5 and FFbH-R, whereas none were found for HPA. Conclusion As expected, a difference in mental health parameters was found for the “known diagnosis” and “no diagnosis+CRP negative” groups. The results also show differences for daily functional tasks between all three groups. However, the lowest scores were found for the group “no diagnosis+CRP pos”, i.e. people with strong indications for an early rheumatic disease. This further supports evidence of reduced functionality for early stage patients and the need for early diagnosis and therapy. The lack of significant differences for HPA patterns could be caused by generally very low physical activity for all three groups. References [1] Baecke JA, Burema J, Frijters JE (1982) A short questionnaire for the measurement of habitual physical activity in epidemiological studies. Am J Clin Nutr 36:936-942. [2] Branco JC, Rodrigues AM, Gouveia N, et al. (2016) Prevalence of rheumatic and musculoskeletal diseases and their impact on health-related quality of life, physical function and mental health in Portugal: results from EpiReumaPt- a national health survey. RMD Open 2:e000166. [3] Triantafyllias K, Leis R, Dreher M, et al. (2019) Depressive Symptomatik bei fruher rheumatoider Arthritis. Zeitschrift fur Rheumatologie (in press). Disclosure of Interests Matthias Dreher: None declared, Julia Sauer: None declared, Nicolas Scharm: None declared, Thomas Plagemann: None declared, Gunter Assmann: None declared, Kirsten Hoeper: None declared, Konstantinos Triantafyllias: None declared, Reinhold E. Schmitt: None declared, Andreas Schwarting Grant/research support from: GSK, Pfizer, AbbVie, Novartis, Roche, Speakers bureau: GSK, Novartis

Background Valid assessment of disease activity leads to improvement of long-term outcomes in patients with rheumatoid arthritis (RA) (1). Clinical disease activity assessment tools such as the Disease Activity Score 28 (DAS28) are partially subjective and do not always depict the real inflammatory burden. Ultrasound (US) and Hand-MRI are important diagnostic modalities which can nevertheless be time consuming (US, MRI), expensive (MRI) or usually performed unilaterally (MRI). Thus, further diagnostic tools are needed. Optical spectral transmission (OST) is a new modality able to assess the blood-specific absorption of light transmitted through a tissue promising quantification of inflammation in the finger and wrist joints of RA patients (commercial device: HandScan - Hemics, The Netherlands)(2). Objectives To examine the diagnostic value of OST in detecting inflammation in patients with RA and to evaluate for the first time its relationship with disease activity markers and various anthropometric and epidemiologic patient characteristics. Methods OST-Measurements were performed in 168 RA-patients and 114 healthy controls. The difference between OST in the two groups was statistically examined and subsequently controlled for the effect of possible confounding factors. Moreover, association of OST with clinical, ultrasound and serological RA activity markers was evaluated. Finally, relationship of OST with radiographic joint pathology and various anthropometric and epidemiologic parameters (BMI, hand-size, gender, age) was examined. Results OST was significantly higher in the patients group in comparison to the control group, even after adjustment for the effects of various confounding factors (padj Conclusion OST correlated with serological and clinical disease activity markers as well as with different anthropometric and epidemiological parameters of RA-patients. OST could therefore prove to be a good non-interventional complementary tool to assess RA activity next to well established diagnostic methods such as the US or MRI. References [1] Katchamart W, et al. Systematic monitoring of disease activity using an outcome measure improves outcomes in rheumatoid arthritis. J Rheumatol2010;37:1411–1415. [2] Onna M Van, et al. Assessment of disease activity in patients with rheumatoid arthritis using optical spectral transmission measurements, a non-invasive imaging technique. Ann Rheum Dis2016;75:511–518. Disclosure of Interests Konstantinos Triantafyllias: None declared, Caroline Heller : None declared, Michele De Blasi : None declared, Peter Galle : None declared, Andreas Schwarting Grant/research support from: GSK, Pfizer, AbbVie, Novartis, Roche, Speakers bureau: GSK, Novartis

To the Editor: We have read with great interest the letter of Verhoeven, et al ,1 referring to our recent publication on the diagnostic value of optical spectral transmission (OST) in rheumatoid arthritis (RA).2 In our work we had described for the first time, to our knowledge, that OST values could be influenced not only by disease-associated factors (i.e., inflammatory activity) but also by patient-associated characteristics, such as sex, BMI, and age. …

Ziel des Rheuma-Netzwerks ADAPTHERA („risikoadaptierte Rheumatherapie“) ist es, eine flachendeckende Verbesserung der rheumatologischen Versorgung durch Koordination der Behandlung in einem landesweiten, transsektoralen Netzwerk zu erreichen. Begleitende biomedizinische Forschungsprojekte, Schulungskonzepte sowie der Aufbau eines Rheumaregisters (Daten- und Biomaterialsammlung) sollen daruber hinaus fur eine stetige und nachhaltige Optimierung der Versorgung sorgen. In der Pilotphase 2012–2015 lag der Fokus des als „Landesleitprojekt“ im Rahmen der Initiative Gesundheitswirtschaft Rheinland-Pfalz (RL-P) geforderten ADAPTHERA Netzwerkes auf der Optimierung der Fruhdiagnose der rheumatoiden Arthritis, bei der bekanntermasen ein erhebliches Versorgungsdefizit besteht. Durch die intensive, dauerhafte und koordinierte Kooperation aller Versorgungspartner auf dem Gebiet der Rheumatologie (niedergelassene Hausarzte und Orthopaden, niedergelassene Schwerpunktrheumatologen, das ACURA-Rheumazentrum RL-P, die Universitatsmedizin Mainz, die Rheumaorthopaden sowie die Arbeitsgemeinschaft Rheumatologie RL-P) ist ein bundesweit einzigartiges flachendeckendes Angebot mit nachweisbarer Versorgungsoptimierung in Rheinland-Pfalz entstanden. Unterstutzt wurde das Netzwerk dabei durch die hervorragende Zusammenarbeit mit der Kassenarztlichen Vereinigung RL-P und der Selbsthilfeorganisation Rheumaliga. Die bei Projektstart gesetzten Ziele werden am Ende der Pilotphase erreicht: - deutliche Verbesserung der Fruhdiagnostik der rheumatoiden Arthritis (durchschnittlich 23,7 Tage Dauer bis zur Diagnosestellung durch Rheumatologen) - krankenkassenubergreifender Zugang (unabhangig von einer bestimmten Kasse) - flachendeckend (nachweislich Teilnahme von Hausarzten aus ganz RL-P) - Aufbau von Daten- und Biomaterialiensammlung etabliert als Grundlage fur Biomarkerforschung und Aufbau eines Rheuma-Registers Rheinland-Pfalz.

ObjectivesWe explored the impact of circulating anti-N-methyl-D-aspartate receptor (NMDAR) antibodies on the severity of fatigue in patients with systemic lupus erythematosus (SLE).MethodsSerum samples of 426 patients with SLE were analysed for the presence of antibodies to the NR2 subunit of the NMDAR. In parallel, the severity of fatigue was determined according to the Fatigue Scale for Motor and Cognitive functions questionnaire. In a subgroup of patients with SLE, the hippocampal volume was correlated with the levels of anti-NR2 antibodies. Isolated immunoglobulin G from patients with anti-NR2 antibodies were used for murine immunohistochemical experiments and functional assays on neuronal cell lines. Treatment effects were studied in 86 patients with lupus under belimumab therapy.ResultsWe found a close correlation between the titre of anti-NR2 antibodies, the severity of fatigue, the clinical disease activity index (Systemic Lupus Erythematosus Disease Activity Index 2000) and anti-double stranded DNA antibodies—independently of the presence of neuropsychiatric lupus manifestations. Pathogenic effects could be demonstrated by (1) detection of anti-NR2 antibodies in the cerebrospinal fluid, (2) in situ binding of anti-NR2 antibodies to NMDAR of the hippocampus area and (3) distinct functional effects in vitro: downregulating the energy metabolism of neuronal cells without enhanced cytotoxicity. Treatment with belimumab for at least 6 months affected both the severity of fatigue and the levels of anti-NR2 antibodies.ConclusionThe presence of anti-NR2 antibodies in patients with SLE with fatigue is a helpful diagnostic tool and may offer a major approach in the therapeutic management of this important disabling symptom in patients with SLE.

Macrovascular involvement and cardiovascular (CV) risk has not been sufficiently studied in mixed connective tissue disease (MCTD). In particular, the gold standard assessment method of aortic stiffness carotid-femoral pulse wave velocity (cfPWV) has never been evaluated in patients with this disease. The aims of the present study were therefore to examine cfPWV in MCTD and to evaluate its associations with MCTD-associated markers and traditional CV risk factors.Measurements of cfPWV were performed in 43 MCTD patients and 107 healthy controls. The difference between cfPWV in the two groups was statistically examined and subsequently controlled for the effect of possible confounding factors. The association of cfPWV with MCTD-associated organ involvement, routine laboratory parameters and immunoserological markers was also evaluated. Finally, the relationship of cfPWV with medications and traditional CV risk factors was examined.Adjusted statistical analyses for confounding factors showed significantly higher cfPWV values in MCTD patients in comparison to controls (padj0.001). cfPWV correlated in both the patients and the control group significantly with age (rho=0.69, p0.001 and rho=0.67, p0.001 respectively) and diastolic arterial pressure (padj=0.024 and padj=0.032 respectively). Moreover, cfPWV correlated in the control group with systolic and mean arterial pressure (padj0.001 and p=0.002 respectively). Finally, higher cfPWV values could be documented in the subset of MCTD patients without lung involvement (padj=0.007).Patients with MCTD have significantly higher aortic stiffness and thus CV risk in comparison to controls. Except for the disease itself, age and blood pressure were the main predictors of cfPWV.

Giant cell arteritis (GCA) is one of the most common forms of inflammatory vasculitis in older patients. Because of possible irreversible vision deterioration, a fastest possible diagnosis and therapy is of absolute importance. To date, there are still no reliable data to obtain an initial assessment of the outpatient health care situation of patients diagnosed with GCA in Rhineland-Palatinate.The specialists (neurologists, rheumatologists, ophthalmologists and general practitioners) participating in the statewide rheumatology network ADAPTHERA were questioned with the help of a questionnaire regarding disease frequency, activity, drug therapy and possible comorbidities. In addition, the collected data were compared and supplemented by the ambulatory coding of the Association of Statutory Health Insurance Physicians in Rhineland-Palatinate.Based on the information provided by general practitioners, 272 GCA patients were treated in Rhineland-Palatinate during the survey period. The average duration of the disease until diagnosis was 3.6 (SD ± 4.8) months. Drug therapy in the form of glucocorticoids was in first place followed by methotrexate, acetylsalicylic acid (ASA) and azathioprine. Cardiovascular diseases, chronic pain syndromes, depression, osteoporosis and diabetes mellitus were also described as comorbidities.The majority of patients with GCA are being cared for by general practitioners (GP). Long-term therapy and timely rheumatological co-treatment seem to be problematic. The primary care providers expressed their wishes for rheumatological training and further education measures. In terms of diagnosis and treatment, there is a demand to implement a "vasculitis fast-track" module.

Patients with systemic lupus erythematosus (SLE) are under increased risk for cardiovascular events (CVE) and mortality. Aortic stiffness, as measured by carotid-femoral pulse wave velocity (cfPWV), has been shown to predict CVE and mortality in the general population. The aim of the present study was to examine the factors associated with cfPWV in patients with SLE and to determine differences of SLE patients in comparison to healthy controls.125 patients with SLE and 104 controls were included. Demographic, medication and cardiovascular risk factor data were collected from all participants. Furthermore, clinical and laboratory SLE associated parameters were documented in the patients' group. All subjects underwent measurements of blood pressure and cfPWV.Interestingly, only age (β=0.55; p0.001), mean arterial pressure (MAP) (β=0.29; p0.001) and estimated glomerular filtration rate (eGFR) (β=-0.20; p=0.033) were associated independently with cfPWV in patients with SLE. Moreover, there was no difference of cfPWV between patients with SLE and controls before (p=0.301) and after adjustment for disparities between the groups (p=0.671).Vascular stiffness in patients with SLE seems to be independent from SLE-related factors and from most traditional CVRF and is mainly associated with age, MAP and renal function defined as eGFR. There is an independent correlation between eGFR and cfPWV in a SLE population with a widely normally ranged eGFR. There is no difference of cfPWV between patients with SLE and controls.

Die Nierenbeteiligung bei rheumatologischen Erkrankungen spielt eine grose diagnostische, prognostische und therapeutische Rolle im klinischen Alltag. Bei Vaskulitiden und Kollagenosen ist die Nierenfunktion in die Standarddiagnostik miteinbezogen. Weniger bekannt ist eine Nierenbeteiligung bei der rheumatoiden Arthritis, obwohl eine solche Manifestation nicht selten ist. Die Klinik zeigt, dass neben medikamentos-toxischen Effekten auch echte renale Manifestationen vorkommen. Sekundare Amyloidosen und Vaskulitiden sowie Glomerulonephritiden stehen hier im Vordergrund und konnen in Kombination mit medikamentos bedingten glomerularen und tubulointerstitiellen Schaden zu differenzialdiagnostischen Problemen fuhren.

Das klinische Bild einer vergroserten Glandula submandibularis und/oder einer vergroserten Glandula lacrimalis fuhrt haufig zu differenzialdiagnostischen Schwierigkeiten. Vonseiten der Rheumatologie sollte ein Sjogren-Syndrom ausgeschlossen werden, insbesondere bei Patienten, die uber Xerophthalmie und Xerostomie uber mehr als 3 Monate klagen. Die Autoren berichten im Folgenden uber den Fall eines Patienten, der sich zur Abklarung einer submandibularen Schwellung und Xerophthalmie vorstellte. In enger Zusammenarbeit von Rheumatologen, Hals-Nasen-Ohren-Arzten und Pathologen konnte die Diagnose einer IgG4-assoziierten Sialadenitis (IgG4-assoziierter M. Mikulicz) gestellt werden.

Background Patients with rheumatoid arthritis (RA) are at a higher cardiovascular (CV) risk in comparison to the general population. CV risk associates closely with aortic stiffness. Aim of this exploration was therefore to evaluate aortic stiffness in patients with RA and to examine its association with various RA associated parameters as well as with traditional CV risk factors. Methods Measurements of carotid-femoral pulse wave velocity (cfPWV) were analyzed retrospectively in 38 RA patients and 25 controls. We investigated the statistical difference between cfPWV values in the two groups. Furthermore, we analyzed the associations of cfPWV with laboratory and clinical RA parameters including Disease Activity Score 28 and its components, rheumatoid factor, cyclic citrullinated peptide antibodies, antinuclear antibodies and RA duration. Finally, we explored the relationship of cfPWV with traditional CV risk factors in the RA group. Results cfPWV was not significantly higher in RA patients in comparison to controls in an adjusted statistical model for confounding factors [−0.587 95 % CI (−1.38 to 0.201), p = 0.144]. Among RA patients there was a statistically significant correlation of cfPWV with age (rho = 0.544, p = 0.001) and the count of tender joints [0.051 95 % CI (0.008–0.207), p = 0.034]. Finally, C-reactive protein associated only marginally with cfPWV [0.105 95 % CI (−0.410 to 0.003), p = 0.053]. Conclusions In RA patients the number of tender, rather than swollen joints correlates with stiffness of the aorta, as measured through cfPWV. Therefore, RA associated joint pain might play a role in the development of aortic stiffness and thus increase CV risk.

Background The significance of early diagnosis and early therapy initiation in patients with RA is tremendous. ADAPTHERA is a state wide rheumatology network based in Rhineland-Palatinate, Germany. ADAPTHERA aims to improve RA prognosis and quality of patients` life by reducing the time-to-RA-diagnosis. While there have been a lot of studies regarding quality of life in patients with established RA 1,2 , adequate data concerning very early rheumatoid arthritis (VERA) (disease duration Objectives To study quality of life in VERA patients and to investigate its associations with various RA associated clinical and laboratory parameters. Methods In a two center subgroup analysis from the ADAPTHERA cohort, we studied quality of life indexes such as Hannover Functional Ability Questionnaire (HFAQ), Health Assessment Questionnaire (HAQ), EuroQol-5D-3L (EQ-5D-3L), Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) and World Health Organisation-5 Well Being Index (WHO-5) in patients with VERA. Moreover, we examined the statistical relationship of these indexes with various clinical and laboratory RA associated parameters such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF)-/ cyclic citrullinated peptide antibodies (anti-CCP)-status and Disease Activity Score 28 (DAS28) as well as its components [counts of tender and swollen joints, Visual Analogue Scale (VAS)]. Results 74 Patients (50 females, 24 males) with a median age of 62 years (IQR 49, 71) were identified as VERA patients. 36 of them were anti-CCP positive and 34 RF positive. Median scores for HFAQ, HAQ, EQ-5D-3L, FACIT-Fatigue, and WHO-5 were 75.5 (IQR 61, 92.5), 0.75 (IQR 0.26, 1.25), 0.89 (IQR 0.79, 0.89), 40 (IQR 32, 46) and 60 (IQR 32, 76) accordingly. The results of the statistical associations between these scores and RA associated parameters are seen in Table 1. Conclusions The quality of life indexes HFAQ, HAQ, EQ-5D-3L and WHO-5 are associated with two or more markers of disease activity in patients with VERA. Therefore, improvement of patients9 quality of life is linked to the achievement of disease remission. In contrast, the FACIT-fatigue index seems to be solely associated with arthritis pain intensity. Thus, improvement of fatigue requires an effective pain management strategy. References Gossec L et al., RMD Open. 2015 Apr 2;1(1):e000019. Her M et al., Curr Opin Rheumatol, 2012 May;24(3):327–34. Disclosure of Interest None declared

Objective To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), malignancies, and functional outcome of interstitial lung disease (ILD). Methods (1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared with 7202 anti-PM/Scl−, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case–control study: additional data were collected for 165 anti-PM/Scl+ SSc patients (85 from the EUSTAR registry) and compared with 257 anti-PM/Scl− SSc controls, matched for sex, cutaneous subset, disease duration and age at SSc onset. Results Patients with isolated anti-PM/Scl+, as compared with anti-Pm/Scl−, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). The presence of muscle involvement was associated with a more severe disease phenotype. Although very frequent, ILD had a better functional outcome in cases than in controls. In patients with both anti-PM/Scl and SSc-specific antibodies, a higher frequency of typical SSc features than in those with isolated anti-PM/Scl was observed. Conclusion The analysis of the largest series of anti-PM/Scl+ SSc patients so far reported helps to delineate a specific clinical subset with muscle involvement, cutaneous DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.