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Objective Despite increased physician’s awareness and improved diagnostic and serological testing in the recent years, the interval between the initial symptoms and the diagnosis of Systemic lupus erythematosus (SLE) is still very long. Our aim was to study this delay and its association to the outcome of the disease. Methods Information on demographics, onset of first symptoms, first physicians visit and time of diagnosis was assessed by self-reported questionnaires among SLE patients in Germany (LuLa cohort, n = 585) in the year 2012. Disease activity (Systemic Lupus Activity Questionnaire; SLAQ), disease related damage (Brief Index of Lupus Damage; BILD), health related quality of life (Short Form 12) and fatigue (FSS) were chosen as proxies for outcome. Linear regression analysis was used to analyze the association of the delay in diagnosis to the outcome, adjusted for age, disease duration and sex. Results Mean duration between the onset of symptoms and the diagnosis of SLE was 47 months (SD 73). The longer the time to diagnosis, the higher the disease activity (β = 0.199, p Conclusion In systemic lupus erythematosus, longer time to diagnosis was associated with worse outcome. Concepts in care with the intention to shorten the time to diagnosis are needed to improve the long-term outcome of the disease.

Objective Our aim was to study the quality of medical care in patients with systemic lupus erythematosus (SLE) to understand gaps and to analyze the association with outcome of the disease. Methods Information on demographics and medical care was assessed by self-reported questionnaires among SLE patients (LuLa cohort, 2011, n = 580). In total, 21 aspects of medical care were analyzed. Univariate analysis selected 10 predictor variables for further analysis: (1) urine examination and (2) blood test in the previous year, (3) taking antimalarials, (4) taking vitamin D and calcium if the dosage of prednisolone was greater than 7.5 mg/day, counseling regarding (5) lipid metabolism, (6) vaccination, and (7) blood pressure, and treatment of the comorbidities (8) hypertension, (9) osteoporosis and (10) lipid metabolism disorder. The association of these 10 items with the outcome of the disease, assessed in 2015, was analyzed by linear regression analysis, adjusted for age, disease duration and sex. Results On average six of the 10 items were met (±1.7). Receiving more clinical care in 2013 was predictive for low disease activity (SLAQ, p = 0.024, β = –0.104, corr. R2 = 0.048), low progress in disease-related damage (Delta Brief Index of Lupus Questionnaire, p = 0.048, β = –0.132, corr. R2 = 0.036) and high health-related quality of life (SF-12 physical, p = 0.035, β = 0.100, corr. R2 = 0.091) in 2015. Conclusion Our study illustrates a link between the quality of care and the SLE outcome parameters disease activity, disease-related damage and quality of life. Consistent considerations of these care parameters, which are recommended in several management guidelines, could therefore be a good approach to improve the outcome of patients with SLE.

Objective The aim of this study was to identify factors associated with impaired work productivity and impaired daily activities in patients with systemic lupus erythematosus (SLE). Methods The LuLa study is a longitudinal patient-reported study. Beyond sociodemographic data, work productivity, daily activities and fatigue, several other clinical outcome parameters (e.g. mental health–related quality of life and physical functioning, disease activity, damage and pain) were surveyed with validated questionnaires. The effects of confounders on work productivity (WPAI 2) and daily activity domains (WPAI 4) were studied by multivariate regression analysis. Results A total of 585 patients completed the questionnaire of whom 259 were employed and analysed. The median impairment in work productivity (WPAI 2) was 20% (Q1–3 0–40), and the median impairment in daily activities (WPAI 4) was 30% (Q1–3 10–50%). Multivariate regression analysis revealed that fatigue, pain, disease activity and health-related quality of life affected WPAI 2 and 4. Furthermore, we observed distinct synergistic effects of fatigue, disease activity and pain on both work productivity and daily activities: a higher impact of fatigue was associated with the reported extent of pain or disease activity. Conclusion In employed patients with SLE, impaired work productivity and impaired daily activities were frequently reported. Fatigue, pain, disease activity and health-related quality of life demonstrated a detrimental impact, with a synergistic effect of fatigue, disease activity and pain. Hence, both optimized pain management and targeted immunomodulatory therapy are important for preserving active participation in life among patients with fatigue.

BackgroundPatients with Systemic Lupus Erythematosus (SLE) experience both physical and psychosocial restrictions that negatively impact their quality of life. Coping mechanisms have turned out to be important contributors to health-related outcomes, not only in SLE but also other chronic conditions. However, there is limited understanding of factors that enhance or hamper coping in SLE.ObjectivesTo analyse parameters associated with longitudinal changes in coping behaviour in patients with SLE who take part in a long-term SLE patient cohort study.MethodsSince 2001, the German nationwide SLE patient longitudinal (Lupus Langzeit ‘LuLa’) study annually administers self-reported questionnaires to SLE patients.In addition to demographic and the annually probed extended clinical data (e.g., medication, disease activity, fatigue, depression), in both 2009 and 2014 we assessed the Pain-related Self Statements Scale (PRSS) to perceive information related to coping behaviour. This includes ‘positive coping’ as well as catastrophizing as a dysfunctional form of coping behaviour.Statistical analysis was accomplished by a linear regression model adjusting for age, pain, number of comorbidities and net income. The PRSS score difference from 2009 and 2014 was used as the dependent variable. Factors from the i) medical (involvement of the skin, pain, fatigue), ii) activity (basic, leisure, sports), iii) intrinsic (depression, kinesiophobia, perceived health control), and iv) social participation categories were entered as independent variables.Results272 patients (96.7% female) provided valid PRSS questionnaires in both 2009 and 2014. In 2009 the mean age in this cohort was 51 years (SD 11.2) with an average disease duration of 16.2 years (SD 8.3). The mean reported lupus activity (VAS 0-10) during the last three months was 3.7 (SD 2.4). During the six-year observation period, the proportion of improvement (46.2%) and deterioration (47.4%) in coping score was almost balanced, while in catastrophizing score more participants improved (50.0%) than deteriorated (37.0%).A perceived high internal control, thus the belief that health outcomes are contingent on personal behaviour, was associated with an improvement in the coping score [HLC, b=0.061 (95%-CI 0.014; 0.109), p=0.012]. Conversely, high external control convictions, e.g., the belief that doctors and other third parties determine health outcomes, were associated with a worse coping score [HLC, b=-0.090 (95%-CI -0.154; -0.026), p=0.006]. Deterioration in depression [ADS-L, b=0.015, (95%-CI 0.006; 0.025), p=0.002] and impaired social participation [IMET, b=0.043, (95%-CI 0.000; 0.085), p=0.050] were associated with a deterioration of catastrophizing, whereas better internal control [HLC, b=-0.046, (95%-CI -0.080; -0.012), p=0.009] was associated with its improvement.Mucocutaneous involvement, fatigue and the extent of physical activity were not significantly associated with either coping or catastrophizing scores in the regression analysis.ConclusionIn line with data from other chronic diseases, our findings in a longitudinal SLE cohort emphasise the role of intrinsic factors, such as mental health status and self-efficacy, improving the quality of life in SLE patients via successful coping behaviour. Affirmative action measures and programs to improve social participation may yield additional benefits.AcknowledgementsThe LuLa study is supported by unrestricted grants from GlaxoSmithKline, UCB Pharma and AstraZeneca.Disclosure of InterestsEmily Reutemann: None declared, Ralph Brinks: None declared, Jutta G. Richter Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Rebecca Fischer-Betz Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Borgi Winkler-Rohlfing: None declared, Martin Aringer: None declared, Matthias Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Gamal Chehab Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study.

Purpose Our aim was to study the delay from the onset of symptoms to the diagnosis of systemic lupus erythematosus (SLE) and its association to the outcome of the disease. Methods Information on demographics, onset of first symptoms, first physicians visit and time of diagnosis was assessed by self-reported questionnaires among SLE patients in Germany in 2010 (LuLa cohort, n=585). Disease activity (Systemic Lupus Activity Questionnaire; SLAQ), disease related damage (Brief Index of Lupus Damage; BILD) and health related quality of life were chosen as relevant proxies for outcome. Linear regression analysis was used to analyze the association to the outcome of the disease, adjusted for age, disease duration and sex. Results Mean reported duration between the onset of symptoms and the diagnosis of SLE was 45.7 months (SD 72.6), including a mean duration of 13.2 month (SD 40.9) between the onset of symptoms and the first physicians visit. In our cohort, the mean disease duration was 17.7 years (SD 7.89). A delayed diagnosis was associated with high disease activity (SLAQ, p Conclusion The time to diagnosis was associated with a worse outcome in systemic lupus erythematosus, assessed by self-reported questionnaires for disease activity, disease-related damage and quality of life, unaffected by the organ involvement at the time of diagnosis. An early diagnosis should therefore be strived to improve the long-term outcome of the disease. German Clinical Trial Register, www.germanctr.de, DRKS00011052 Acknowledgement The LuLa study is supported by unrestricted grants from GlaxoSmithKline and UCB Pharma.

Objective The European League Against Rheumatism and the American College of Rheumatology jointly embarked on a new classification criteria for systemic lupus erythematosus (SLE) project. Its first phase involved generation of a broad set of items potentially useful for classification of SLE. This study was undertaken to add the patient perspective to an expert Delphi approach and an early patient cohort study. Methods A national cross-sectional study was conducted. A self-report questionnaire was published in the “Schmetterling” (Butterfly), the quarterly journal of the German SLE patient association. Individuals with SLE were asked to anonymously complete the questionnaire, which asked for demographic details, organ manifestations, autoantibodies and symptoms. Results A total of 339 completed questionnaires out of 2498 were returned, a response rate of 13.6%; 83.2% reported they were ANA positive and 81.7% reported joint, 66.1% skin and 33.0% renal involvement. For the time before and in the first year after their SLE diagnosis, the majority reported fatigue (89.4%), joint pain (86.7%), photosensitivity (79.4%) and myalgia (76.1%). Of interest, more than half of the patients reported fever as an early symptom (53.7%). Conclusion For a Caucasian European SLE patient population, the overall characteristics suggest meaningful representation. While many symptoms were reported as expected, the high percentage of patients reporting fever and the significant number of patients with unexpected gastrointestinal complaints are of particular interest. These data add to the information on early SLE symptoms informing the development process of new SLE classification criteria.

Objectives Vaccinations are an important measure to prevent infections in immunocompromised patients. The knowledge of vaccination coverage and reasons for non-vaccination in patients with SLE is scarce. The aim of this study was to assess coverage rates of selected vaccinations in a representative sample of SLE patients and to identify predictors for non-vaccination. Methods In 2013, information on selected vaccinations (coverage, application and reservations) and on demographics, clinical parameters and health beliefs was assessed by means of a self-reported questionnaire among a representative sample of SLE patients in Germany (LuLa cohort). Results Five hundred and seventy-nine patients participated. Vaccination status was primarily checked by their general practitioner (57.3%). Of all the patients, 24.9% did not get their vaccination status checked at all, 16.1% had generally been advised against the use of vaccinations by a physician, and 37.5% stated that they had rejected vaccinations themselves. Their main reasons were fears of developing a lupus flare (21.8%) or adverse events (13.5%). A greater belief by patients in the doctor controlling one's health and the general benefit of medication prevented the rejection of vaccines. Vaccination coverage was low for all recorded vaccinations (tetanus 65.8%, influenza 45.2%, pneumococcus 32.2% and meningococcus 6.1%). Older age was predictive of receiving influenza and pneumococcal vaccination. The same applies for CSs >7.5 mg for receiving influenza vaccination. Conclusion Vaccination coverage in SLE patients is poor and reflects insufficient implementation of national and international recommendations. Rheumatologists need to recognize patients' reservations against vaccinations, to communicate their importance and safety and to give individual recommendations to patients and their health-care providers. Trial registration German Clinical Trials Register, www.germanctr.de, DRKS00011052.

Background Systemic lupus erythematosus (SLE) is a chronic disease, which is still associated with significant morbidity and mortality1. Recommendations for the management of SLE patients exist2-5, but information on its implementation and the resulting impact on long-term outcome remain unclear. Objectives Our aim was to study the quality of SLE medical care in Germany to understand gaps and to analyze the association to long-term outcome parameters. Methods In the LuLa-study information on demographics, clinical and medical care parameters are assessed annually by self-reported questionnaires among a representative sample of SLE patients in Germany (LuLa cohort, n=572). In 2013 additional questions on the management of care, as mentioned in current guidelines and recommendations, were surveyed. Ten items predicting a good clinical care (quality measures) were evaluated and an overall score with a minimum of 0 points and a maximum of 10 points was calculated. The ten items are taking antimalarials, osteoporosis protection at a dose above 7.5mg prednisolone equivalent per day or taking ≤ 7.5mg per day, vaccination, blood pressure, fat metabolism counseling, urine examination and blood test once a year, treatment of comorbidity fat metabolism disorder, osteoporosis and hypertension. Health related quality of life (Short Form Survey, SF-12/36), damage (Brief Index of Lupus Damage, BILD) and disease activity (Systemic Lupus Activity Questionnaire, SLAQ) were chosen as relevant proxies for long-term outcome. Using linear regression, we examined the relationship between the quality measures and outcome parameters, adjusted for age, disease duration and gender. Results 65.5% of the patients consulted a rheumatologist as a main contact for their disease. On average 6.1 points of the 10 quality measures were met (SD 1.7). The fulfillment of these quality measures varied between 22.8% (fat metabolism counseling) and 97.6% (osteoporosis protection at a dose above 7.5mg prednisolone equivalent per day or ≤ 7.5mg per day). Fulfilling more clinical care items in 2013 was predictive for high disease-related quality of life (SF-36, p=0.004), low progress in disease-related damage (BILD, p=0.048) and low disease activity (SLAQ, p=0.046) in the subsequent years. Conclusion Our study illustrates a strong link between the quality of care and important SLE outcome parameters: quality of life, disease-related damage and disease activity assessed by a self-reported questionnaire. Consistent considerations of these care parameters, which are recommended in several management guidelines, yield a positive effect on outcome. An interdisciplinary approach with general practitioners and other specialists is certainly beneficial and warranted. German Clinical Trial Register, www.germanctr.de, DRKS00011052 References [1] Borchers, A. T., Keen, C. L., Shoenfeld, Y. & Gershwin, M. E. Surviving the butterfly and the wolf: mortality trends in systemic lupus erythematosus. Autoimmun. Rev. 3, 423–453 (2004). [2] Bertsias,G. et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann. Rheum. Dis. 67, 195–205 (2008). [3] Mosca, M. et al. European League Against Rheumatism recommendations for monitoring patients with systemic lupus erythematosus in clinical practice and in observational studies. Ann. Rheum. Dis. 69, 1269–1274 (2010). [4] Guidelines for referral and management of systemic lupus erythematosus in adults. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Arthritis Rheum. 42, 1785–1796 (1999). Disclosure of Interests Anna Kernder Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Jutta Richter Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Rebecca Fischer-Betz Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Borgi Winkler-Rohlfing: None declared, Matthias Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Speakers bureau: Chugai, Gamal Chehab Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study.

Background:Patients with systemic lupus erythematosus (SLE), even compared to those with other rheumatic diseases, are thought to often experience a long delay from the onset of symptoms to diagnosis.Objectives:Our aim was to study the association of this delay to the long-term outcome of the disease.Methods:Information on demographics, onset of first symptoms, first physicians visit, time of diagnosis and organ involvement was assessed by self-reported questionnaires among SLE patients in Germany in 2011 (LuLa cohort, n=585). Disease related damage (Brief Index of Lupus Damage; BILD), disease activity (Systemic Lupus Activity Questionnaire; SLAQ) and health related quality of life (SF-12) were chosen as proxies for outcome. The association to the outcome of the disease was analyzed by linear regression analysis, adjusted for age, disease duration, organ involvement and sex.Results:In our cohort, the mean reported duration between the onset of symptoms and the diagnosis of SLE was 46 months (SD 73), which includes 13 months (SD 41) between the onset of symptoms and the first physicians visit. The participating patients were diagnosed between 1960 and 2004.Linear regression analysis revealed that longer time to diagnosis was associated with (i) higher disease activity (SLAQ, pConclusion:A delay in diagnosis was associated with a worse outcome in SLE (disease activity, disease-related damage and health-related quality of life). Therefore, an early diagnosis seems to be important to improve the long-term outcome of the disease. It will be interesting to see whether adopting the new EULAR/ACR 2019 classification criteria can contribute to a faster diagnosis and a better outcome in consequence.The LuLa study is supported by unrestricted grants from GlaxoSmithKline and UCB Pharma.Disclosure of Interests:Anna Kernder Grant/research support from: Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Jutta Richter Grant/research support from: Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Rebecca Fischer-Betz Consultant of: UCB, Speakers bureau: Abbvie, Amgen, Biogen, BMS, Celgene, Chugai, GSK, Janssen, Lilly, Medac, MSD, Novartis, Roche, UCB, Pfizer., Borgi Winkler-Rohlfing: None declared, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche, Matthias Schneider Grant/research support from: GSK, UCB, Abbvie, Consultant of: Abbvie, Alexion, Astra Zeneca, BMS, Boehringer Ingelheim, Gilead, Lilly, Sanofi, UCB, Speakers bureau: Abbvie, Astra Zeneca, BMS, Chugai, GSK, Lilly, Pfizer, Sanofi, Gamal Chehab Grant/research support from: Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study.

Objective Adherence to medication has a major impact on treatment control and success especially in chronic diseases but often remains unrecognized. Besides clinical, socioeconomic, disease-related and treatment-related parameters, general and personal health beliefs, as well as perception of health, can affect adherence. Our aim was to investigate the adherence to lupus-specific medications in German lupus patients and to assess influencing factors including detrimental or beneficial effects of health perceptions and beliefs. Methods The Lupus Erythematosus (LE) Long-Term Study (LuLa-study) is a nationwide longitudinal study among German Caucasian patients with systemic lupus erythematosus who have been assessed annually using a self-reported questionnaire since 2001. In 2013, we included questions concerning medical adherence (Morisky Medication Adherence Scale; MMAS-4), beliefs about medication prescribed (BMQ), illness perception and about the patients’ health locus of control (HLC). We present a cross-sectional analysis to assess predictors of adherence using a multivariable stepwise logistic regression. Results Five hundred and seventy-nine patients participated, 81 of whom did not take any lupus-specific medication and 40 of whom did not complete the MMAS-4 and were therefore omitted. Only 62.7% reported high adherence. Unintentional behaviour for low medical adherence exceeded the intentional behaviour by far. The use of azathioprine (OR: 1.85; 95% CI: 1.02–3.34), prednisone Conclusion A low belief that one's own health is determined by healthcare providers (external HLC) and the belief of the harmfulness of medication were independent predictors of low adherence besides age and the choice of the medical agent. The recognition of these potential obstacles in physician–patient relationships is essential to ameliorate adherence. Provision of sufficient information and education might help to reach the best possible outcome.

Background Participation of chronically ill patients can be permanently and severely impaired. In a heterogeneous disease like systemic lupus erythematosus (SLE), various influencing factors such as disease activity, damage, concomitant diseases, but also detrimental effects due to the psychological burden must be considered. Objectives Our objective was to assess the current state of participation in a representative sample of German patients with SLE and evaluate the impact of demographic and clinical factors. Methods The Lupus erythematosus long-term study (LuLa-Study), a nationwide longitudinal study among German Caucasian patients with SLE, is being conducted annually by a self-reported questionnaire since 2001. Inclusion criteria are a diagnosis of SLE and returning the completed paper questionnaire. Amongst others medication, comorbidities, health-related quality of life (HRQoL, Short-Form-12), and disease activity (Systemic Lupus Activity Measure) are surveyed. In the year 2015 we additionally inquired about participation using the ”Index zur Messung von Einschrankungen der Teilhabe” (IMET; Index for measuring limitations of participation) that was developed on the basis of the International Classification of Functioning, Disability and Health (ICF), as well as depression (Centre for Epidemiologic Studies Depression Scale), and pain coping (Pain Related Self statements scale). A multiple linear regression was run to predict overall impairment and impairment in the individual subdomains (dependent variables). Age, disease duration, number of comorbidities, pain, disease activity, catastrophizing, coping, depression, physical functioning, and physical and mental HRQoL were entered into our model as candidates for the independent variables. Variable selection was accomplished by a stepwise approach based on Akaike information criterion (AIC). Results The questionnaire was completed by 579 patients (response rate 89.2%). Only 48 (8.3%) reported no impairment of participation by their disease. Most limitations were reported in the domains ‘stress and extraordinary strains’ (56.3% reported moderate to high impairment) and ‘sex life’ (48.7%), whereas ‘common activities of daily life’ (21.1%) and ‘close personal relationships’ (26.5%) seemed to be limited less frequently. Depression, physical functioning, and physical and mental HRQoL predicted overall participation (F-test, p Depression, physical functioning, and physical HRQoL predicted also most of the participation subdomains whereas age, disease duration, no. of comorbidities, disease activity, pain, and pain coping behaviour impacted only individual subdomains. Conclusions Limitations of participation are common in SLE patients and affect different areas of life. In order to improve participation, it is of great importance to maintain, respectively improve physical and mental quality of life, physical functioning, and depression. The direction of causality cannot be proved beyond reasonable doubt in this cross-sectional analysis. Additional longitudinal studies are necessary. Acknowledgements The LuLa-study is supported by unrestricted grants from GlaxoSmithKline and UCB Pharma. Disclosure of Interest G. Chehab Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., J. Richter Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., R. Fischer-Betz Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., R. Brinks: None declared, B. Winkler-Rohlfing: None declared, M. Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study.

Background Fatigue, defined as loss of vitality, is a major burden in patients with systemic lupus erythematosus (SLE). It is well-known from cross-sectional studies that fatigue is associated with physical functioning (PF) whereas the effect of fatigue on subsequent PF has not yet been further looked on. Methods Data from the German Lupus-Langzeitstudie (LuLa) cohort was used to explore the effect of self-reported vitality on PF. The ongoing LuLa study comprises patients from the German SLE self-help group. The current analysis uses data from 2002 to 2013. We looked for associations between the PF score (outcome) and the vitality score of the SF-12 questionnaire in one to four years preceding the outcome. Statistical analysis was accomplished by a linear mixed regression model (LMM) adjusting for the potential confounders age, sex, disease duration and disease activity as surveyed by the Systemic Lupus Activity Questionnaire score (SLAQ). Results 1511 patients (93.3% women) provided data with a total of 7841 person-years of observation. Median age at study entry was 46 (interquartile range: 37 to 57). The table 1 shows the regression coefficients of the vitality score (and 95% confidence intervals, CI) estimated by the LMM without and with adjusting for age, sex, disease duration and SLAQ. Conclusions We found evidence that self-reported vitality in patients with SLE is associated with PF in the subsequent years. The data indicate a “window of opportunity” for treating fatigue symptoms of up to four years for maintenance of PF. Disclosure of Interest J. Mucke: None declared, G. Chehab Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, R. Fischer-Betz Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, J. Richter Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, B. Winkler-Rohlfing: None declared, M. Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, R. Brinks: None declared

Introduction Mental symptoms are a major problem in patients with systemic lupus erythematosus (SLE). From geriatric patients it is well-known that prevention of mental disorders leads to maintenance of physical functioning (PF). Methods Data from the German Lupus-Langzeitstudie (LuLa) open cohort was used to explore the effect of self-reported mental HRQoL on PF. The ongoing LuLa study comprises patients from the German SLE self-help group. The current analysis uses data from 2002 to 2013. We looked for associations between the PF score (outcome) and the mental component summary (MCS) score (exposure) of the SF-12 questionnaire in one to four years preceding the outcome. Statistical analysis was accomplished by a linear mixed regression model (LMM) adjusting for the potential confounders age, sex, disease duration and disease activity as surveyed by the Systemic Lupus Activity Questionnaire score (SLAQ). Results 1511 patients (93.3% women) provided data with a total of 7841 person-years of observation. Median age at study entry was 46 (interquartile range: 37 to 57). Table 1 shows the regression coefficients of MCS (and 95% confidence intervals, CI) estimated by the LMM without and with adjusting for age, sex, disease duration and SLAQ. Conclusions We found evidence that self-reported mental HRQoL in patients with SLE is associated with PF in the subsequent years. The data indicate a ‘window of opportunity’ for treating mental symptoms of up to three years for maintenance of PF.

Supplemental material for Early symptoms of systemic lupus erythematosus (SLE) recalled by 339 SLE patients by N Leuchten, B Milke, B Winkler-Rohlfing, D Daikh, T Dörner, S R Johnson and M Aringer in Lupus

Background Health Locus of Control (HLC) is the degree to which individuals believe that their health is controlled by internal (self-responsibility) or external factors (healthcare professionals or chance). This generalized expectancy might affect different disease aspects, especially in chronic diseases. Objectives Our objective was to assess the influence of HLC on different disease aspects in a representative sample of German systemic lupus erythematosus (SLE) patients. Methods The LuLa-Study is a longitudinal survey on a multitude of SLE associated factors that is being conducted annually by means of a self-reported questionnaire among members of the German LE self-help community since 2001 and is ongoing. Inclusion criteria are a diagnosis of SLE and returning the completed paper questionnaire. Amongst others medication, health-related quality of life (Short-Form-12), damage (Brief index of lupus damage) and disease activity (Systemic Lupus Activity Questionnaire) are surveyed. In 2013 we additionally inquired about the health locus of control (HLC) that distinguishes between the “internal” HLCint (self-responsibility) and the two external dimensions HLCdoc and HLCchance considering their doctor respectively chance responsible for personal health. A high HLC was assumed for values above the upper quartile of the specific scales. Accessory questions examined fatigue (Fatigue severity scale), medication adherence (Morisky medication adherence scale), and illness perception (Brief illness perception questionnaire). Results Patients with a high internal health locus of control (HLCint 13.1 vs. 9.1) had less pain (numeric rating scale 0–10), less flares, a better mental and physical health related quality of life, lower disease activity and less fatigue. Patients with a high external 9doctor9-related health locus of control (HLCdoc 10.7 vs. 7.2) were older, had more co-morbidities, more disease damage and received more frequently an immunosuppressive therapy. No significant differences were found between the patients with a high external 9chance9-related HLC compared to the lower scoring patients (HLCcha 11.1 vs. 6.3). Participants with a high external 9doctor9-related HLC had a more threatening view on their illness and a better adherence to medication (high adherence in 78.6% vs. 59.4%). Participants with a high internal HLC perceived their disease significantly less threatening. Higher education levels (school education, further education) went along with a decrease of external 9doctor9-related HLC (HLCdoc). Conclusions Health locus of control has a significant impact in patients with SLE. Depending on the HLC different disease characteristics, treatments, levels of medication adherence and illness perception were noticed. Holistic care needs to consider the impact different HLCs may have. The direction of causality cannot be proved beyond reasonable doubt in this cross-sectional analysis. Hence additional longitudinal studies are necessary. Acknowledgements The LuLa-study is supported by unrestricted grants from GlaxoSmithKline and UCB Pharma. Disclosure of Interest G. Chehab Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, J. Richter Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, R. Brinks: None declared, R. Fischer-Betz Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study, B. Winkler-Rohlfing: None declared, M. Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study

Objective The aim of this study was to identify factors associated with pain coping and catastrophising in patients with systemic lupus erythematosus. Methods All patients were participants of the lupus erythematosus long-term study, which is based on patient-reported data assessed among members of the German Lupus Erythematosus Self-Help Organization. Assessments were performed by means of a questionnaire. Among self-reported clinical data the Pain-Related Self Statements Scale (PRSS) was included. To depict significant differences univariable analyses were carried out using non-parametrical rank tests. To examine factors influencing our outcome variables, we performed a multivariable stepwise regression model including variables that presented significantly in the univariable analysis. Results 447 cases (94.9% female) were analysed showing a mean catastrophising score of 1.1 (SD 0.8) and a mean coping score of 2.8 (SD 0.9) in the PRSS subscales. Higher catastrophising quartiles went along with higher experienced pain, lupus activity, fatigue, damage and decreased health related quality of life, whereas they presented inversely for coping. In our multivariable model, factors associated with catastrophising were: number of lupus-specific drugs (p value 0.004), pain in the last 7 days (p value 0.034), the Short Form 12 Health Survey Mental Component Summary (p value

During the last few decades, the prognosis for patients with systemic lupus erythematosus (LE) has changed from high early mortality to a more chronic longterm course. Although the prevalence of LE has been estimated at 20-50/100,000, data concerning the situation of LE patients in Germany are sparse. Since 2001, a documentation within the German Lupus Self-Help Organisation scheduled for a period of 10 years (LULA) has been recording at the patient level the actual status and the long-term course of a large group of LE patients. A questionnaire adapted from the German rheumatological database is updated once a year and sent to all members.In 2001, 1033 members participated in the documentation. Of these, 92.2% were women (mean age 45.8 years) with a mean disease duration of 9.9 years. 37.6% were employed, and 24.5% were on early retirement. 50.2% rated their overall health status as "not so good" or "poor". Most were receiving treatment with [hydroxy-]chloroquine (35.2%) or azathioprine (21.9%), while 67.9% were receiving corticosteroids. The most frequent comorbidities reported were hypertension (33%), scarring skin disease (24.4%), osteoarthritis (25.2%), osteoporosis (24%), psychiatric disorders/depression (22.9%) and chronic renal disease (22%). Thromboembolic events were reported in 18.5%, myocardial infarction in 2.3% and stroke in 4.8% of cases. Concerning their main contact person for health care, 63.6% specified the rheumatologist. In comparison with other cohort studies and in particular with the German rheumatological database, the data provided exclusively by patients are feasible. Concerning the severity of their disease, their treatment and their global assessment of health status, LULA participants are comparable with other LE patients and can be seen as representative of LE patients in Germany. Further assessment especially of long-term data are needed to obtain additional insights into the burden of the disease and the need for special medical care.

Background EULAR and ACR have jointly funded a project to develop systemic lupus erythematosus (SLE) classification criteria, aiming at earlier and more accurate classification of the disease. This abstract reports on an early phase of that project, aimed at collecting potential candidate criteria. Since SLE patients usually experience the onset and diagnosis as a critical life event, memories of this time appear remarkably accurate. Objectives The objective of this study was to identify early symptoms of SLE from the patient perspective. Methods As approved by the local ethics committee, we conducted a cross-sectional survey of German SLE patients. An anonymous self-report questionnaire was published in the “Schmetterling” the quarterly journal of the “Lupus erythematodes Selbsthilfegemeinschaft”, the German SLE patient association. Patients were asked for year of and age at their initial diagnosis. The questionnaire included typical organ manifestations, symptoms and autoantibodies. In addition, patients were asked to add additional symptoms in free text. For each symptom, boxes were provided to indicate the presence of the symptom before diagnosis, in the first year of diagnosis and at the time of completion of the questionnaire. Results 333 patient questionnaires were completed and mailed. Of the respondents, 93% were female. The respondents9 mean age at diagnosis was 36 years, their mean disease duration was 17 years. 83% reported to be definitely ANA-positive. Joint, skin and kidney involvement were reported at 82%, 66% and 33%, respectively. 22% of patients reported fibromyalgia. For the time before and shortly after diagnosis, more than 50% of the patients reported fatigue (89%), joint pain (87%), sensitivity to sunlight (79%), myalgias (76%), rash (71%), fever (54%), Raynaud9s (52%) and hair loss (51%). Approximately one third (37%) affirmed shortness of breath. Free text symptoms reported frequently before or early in diagnosis are listed in Table 1. Conclusions For a Caucasian European SLE patient population, the overall characteristics suggested meaningful representation. While many symptoms were reported as expected, a significant number of patients volunteered gastrointestinal complaints and symptoms of the central and peripheral nervous system. Solicitation of self reported information from the patient perspective has not been widely used in the development of classification criteria for SLE. These data add to the information on early SLE symptoms that may help improve classification of early SLE. Disclosure of Interest None declared

The aim of this longitudinal study was to determine disease-specific and individual factors associated with health-related quality of life (HRQOL) in a cohort of patients with systemic lupus erythematosus (SLE) organized in the German Lupus Erythematosus Self-Help Organization. Three hundred and seventeen patients aged between 11 and 77 years participated annually in five surveys carried out between 2001 and 2005. Regression analyses were carried out for physical and mental HRQOL as dependent variables. Factors influencing HRQOL were the respective HRQOL scores of the previous year, SLE activity as measured by the Systemic Lupus Activity Questionnaire (SLAQ), and impairments in everyday life. Social support indicated by living in marriage or in a marriage-like partnership had a positive influence on both mental and physical HRQOL, whereas individual factors such as education seemed to be of minor importance.

Background Medical adherence is an important factor influencing therapeutic success. In the majority the lack of adherence remains unrecognised and/or is misinterpreted as non- respectively not adequate response. In Germany and several other countries medical costs are paid by a social health insurance system. Therefore, a lack of adherence with disposal of the unused drugs has a prejudicial effect on national economies. Objectives Our objective was to investigate the frequency and influencing factors of low adherence in lupus patients in Germany. Methods The LuLa-Study is a longitudinal cross-sectional survey of the German LE self-help community on a multitude of LE associated factors. It is being conducted annually by means of a self-reported questionnaire since 2001. Amongst others health-related quality of life is measured by the Short-Form-12 (SF-12) with its mental (MCS) and physical component summary (PCS), damage by using the “Brief Index of Lupus Damage” (BILD) and disease activity using the “Systemic Lupus Activity Questionnaire” (SLAQ). In 2012 we included additional questions concerning medical adherence (using the four item Morisky Medication Adherence Scale; MMAS-4) as well as satisfaction (numeric-rating-scale; 0-10) concerning their lupus medication (i. a. antimalarials, glucocorticoids, immunosuppressive agents). Results In 2012 we received 579 questionnaires. The MMAS-4 was completed by 458 participants. 81 individuals did not currently take any lupus medication and in 40 cases the MMAS-4 was incomplete and therefore omitted. 62.7% showed a high, 32.5% a moderate and 4.8% a low adherence. Increasing age (p Conclusions Our reported adherence rates lie within the large range reported by the review of Costedoat-Chalumeau et al [1]. We disclose several aspects that need to be taken into account when evaluating and initiating a therapy in patients with lupus. Especially in younger patients and those with a worse mental status lower medical adherence should be considered. The choice of the medical agent might have an additional negative impact on patients9 adherence. When initiating a therapy, potential obstacles should be addressed and discussed openly between patients and physicians. Thus, individual therapy planning may contribute to improved adherence and thus produce the best possible outcome. References Adherence to treatment in systemic lupus erythematosus patients. Costedoat-Chalumeau et al. Best Pract Res Clin Rheumatol. 2013 Jun;27(3):329-40. Acknowledgements The LuLa-study is supported by unrestricted grants from GlaxoSmithKline and UCB Pharma. Disclosure of Interest None declared

Morbidity and co-morbidities in patients with lupus erythematosus (LE) result in an impairment, limiting activities of daily living. We compared the handicap, appraised by the German welfare system in patients with LE and their diagnosis, morbidities and co-morbidities, therapy, functional capacity, psychological status and general health. The data were obtained in an anonymous self-administered questionnaire conducted by the lupus patient association. 892 data sets were available for the trial. The mean age was 46.9y, disease duration was 10.3y. The patients had 3.8 concomitant diseases on average. 39.7% were still working and 35.4% drew a pension. 59.2% made an application for disability, which wasn't assigned in only 2.5% of cases. 82.6% had an average grade of disability of more than 50%. It demonstrated a good correlation to age, disease duration, type of LE and therapy. Predictors for a worse assessment were disease duration, osteoarthritis, cerebral insults and functional capacity. In conclusion the data demonstrate a fair appraisal of disability by the German welfare system in patients with LE. Self-administered questionaires can yield credible results.

During the last few decades, the prognosis for patients with systemic lupus erythematosus (LE) has changed from high early mortality to a more chronic longterm course. Although the prevalence of LE has been estimated at 20-50/100,000, data concerning the situation of LE patients in Germany are sparse. Since 2001, a documentation within the German Lupus Self-Help Organisation scheduled for a period of 10 years (LULA) has been recording at the patient level the actual status and the long-term course of a large group of LE patients. A questionnaire adapted from the German rheumatological database is updated once a year and sent to all members.In 2001, 1033 members participated in the documentation. Of these, 92.2% were women (mean age 45.8 years) with a mean disease duration of 9.9 years. 37.6% were employed, and 24.5% were on early retirement. 50.2% rated their overall health status as "not so good" or "poor". Most were receiving treatment with [hydroxy-]chloroquine (35.2%) or azathioprine (21.9%), while 67.9% were receiving corticosteroids. The most frequent comorbidities reported were hypertension (33%), scarring skin disease (24.4%), osteoarthritis (25.2%), osteoporosis (24%), psychiatric disorders/depression (22.9%) and chronic renal disease (22%). Thromboembolic events were reported in 18.5%, myocardial infarction in 2.3% and stroke in 4.8% of cases. Concerning their main contact person for health care, 63.6% specified the rheumatologist. In comparison with other cohort studies and in particular with the German rheumatological database, the data provided exclusively by patients are feasible. Concerning the severity of their disease, their treatment and their global assessment of health status, LULA participants are comparable with other LE patients and can be seen as representative of LE patients in Germany. Further assessment especially of long-term data are needed to obtain additional insights into the burden of the disease and the need for special medical care.

The medical treatment of people with systemic lupus erythematosus has improved considerably during the last decades. Persons with the condition die less and less often because of the condition and death following a severe infection due to immunosuppressive medication has decreased in frequency. However, the number of patients dying because of arteriosclerosis has increased. As lupus patients have a higher risk to develop arteriosclerosis due to their condition and the necessary therapy, it is very important to influence as many risk factors as possible. In order to raise awareness with patients and doctors about the risk factors and possibilities of prevention, the lupus prevention passport has been developed. Using a traffic light code, the risk factors smoking, excess weight, high blood pressure, high glucose level and lack of exercise can be identified and evaluated. The passport contains an additional emergency section and a chart which allows the patient to carry with them their recent data and information about their medication. The use of the passport has been evaluated. The Lupus Erythematosus Self-help Organisation supports the use of the passport through a special internet platform www.lupus-pass.de. Further training was supposed to increase the use of the passport, which has partly succeeded. Disclosure of Interest None Declared

Background Damage represents an important outcome parameter in systemic lupus erythematosus (SLE). Later in disease course it has a major impact on morbidity and mortality. Therefore, the assessment of damage in SLE is an important task. Most prior assessments are physician-reported but recent efforts try to establish patient-reported damage instruments ([1][1], [3][2]). Those are useful when frequent assessments are needed, e.g. in clinical studies, or if medical care is not timely available. Objectives Lately we validated and evaluated the German version of the Brief Index of Lupus Damage (BILD). It showed a comparable validity to the original BILD with even higher correlation to physician-reported damage, even when used as a self-reported questionnaire ([2][3]). To further evaluate the construct validity of the instrument, we sought to examine it in a large observational cohort of SLE-patients. Methods We used the LuLa-Study which is a longitudinal cross-sectional survey of the German LE self help community on a multitude of LE associated factors. It is being conducted annually by means of a self-reported questionnaire since 2001. In 2011 we included the German BILD; in 2009 we had included the SLICC/SDI to be completed by the attending physicians. To evaluate construct validity we examined disease-related outcomes and patient assessments. Additionally, we assessed the comparability of the BILD with the SLICC/SDI. Results Despite a two year interval between the survey of the SLICC/SDI and BILD they still presented a moderately high correlation of r=0.581 (p < 0.001). Both damage scores showed comparable small to medium correlations with other patient assessments and outcomes. As expected the BILD correlated moderately with the number of co-medications reflecting co-morbidities (r=0.477), physical functioning (r=-0.383) and to a lesser extent with disease activity (r=0.337) as well as duration, age, flares, fatigue and SF-12 MCS (all r

Background Morbidity due to high disease activity, damage, as well as co-morbidities in patients with lupus erythematosus (LE) lead to impairments and disabilities, limiting activities of daily living. In Germany compensations for persons with disabilities according to the EU charter of fundamental rights are complex. The prerequisite for compensation is an official governmental approval of the disability which grades severity from 30 to 100%. Objectives The aim was to highlight possible influential factors for limitations and disability in patients with long-standing lupus. Methods The LuLa-Study is a longitudinal cross-sectional survey of the German LE self help community on a multitude of LE associated factors that is being conducted annually by means of a self-reported questionnaire since 2001 and is ongoing. Data on the disability status have been collected in the reference periods 2003 (n=864) and 2009 (n=604) with census of the application for disability, the grade of disability, the year of approval and the retirement because of disabilities from LE or other reasons. Results In 2009 76.3% had submitted an application for disability, 11.0% more than in 2003, of which more than 96.5% were approved (grade of at least 30%). The mean grade increased from 59.0% in 2003 to 61.8% in 2009. 414 pts (66.8% of the 2009 cohort) completed both assessments in 2003 and 2009. Within this 6-year interval 10.9% (n=45) made an application for disability, of which only 3 application were not approved. The mean grade of disability was 50.7±18.5% (30-100), the mean age at receipt was 44.4±15.0 years and the mean disease duration 9.4±6.4 years. When comparing these patients with a new application for disability with those that had never made an application in 2009 (n=156) they showed a lower score in the mental component summary of the SF-12 (SF-12 MCS), a higher number of specific lupus medication and co-medication, a higher disease activity (VAS 0-10) and more damage (SLICC) (all p In 224 patients a 6-year follow-up of disability was possible. 23.7% showed an increase and 2.7% a decrease in the grade of disability whereas the rest remained stable. The mean percentage increase in grade of disability was 20.0%, the mean percentage decrease 11.7%. The group with a percentage increase showed significantly more co-medications and differed significantly in age at approval, with the highest mean age in the group with increased percentage (44.0 years) (31.2 years in the decrease % group, 39.8 years in the stable group). Conclusions Accumulated damage, disease activity, the number of lupus medication and co-medication, and SF-12 MCS appear to play a role in the evaluation and approval of disability, whereas a higher number of co-medications as well as a higher age at approval seem to predict a deterioration of disability over time. Disease control with avoidance of damage and development of co-morbidities might improve quality of life in patients and reduce the economic burden by disabilities. The relationship between the investigated parameters and other influencing factors need further evaluation. References Sander O et al. [Disability in lupus erythematosus]. Versicherungsmedizin. 2006; 58(3):120-5. Fischer-Betz R et al. [Lupus in Germany: analysis within the German lupus self-help organization (LULA)]. Z Rheumatol. 2005; 64(2):111-22. Disclosure of Interest None Declared