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Was ist neu? Planung von Schwangerschaften Fragen der Familienplanung sind ein wichtiger Bestandteil in der Betreuung von Patientinnen mit entzündlich-rheumatischen Erkrankungen. Die Thematik sollte frühzeitig angesprochen werden. Eine sorgfältige Planung führt nachweislich zu mehr erfolgreichen und komplikationsarmen Schwangerschaften. Zunehmend in den Fokus rückt das Screening nach Risikofaktoren für eine Präeklampsie, da sich hieraus die Indikation zu einer prophylaktischen Einnahme von niedrig dosiertem Aspirin ab der Frühschwangerschaft ergeben kann. Neuere Empfehlungen unterstützen dabei praxisnah. Schwangerschaftsverlauf Bei Frauen mit Rheumatoider Arthritis oder Spondyloarthritis, die mit biologischen krankheitsmodifizierenden antirheumatischen Medikamenten (bDMARDs) behandelt werden, sollte eine Fortsetzung der Medikamentengabe während der Schwangerschaft in Betracht gezogen werden, um das Risiko eines Krankheitsschubs und nachteiliger Schwangerschaftsfolgen zu reduzieren. Bei SLE-Schwangerschaften bietet eine HCQ-Therapie mit einer Reduktion des Risikos einer Präeklampsie und eines kongenitalen AV-Blocks einen Benefit, der über die reine Krankheitskontrolle hinausgeht. Therapie vor, in und nach der Schwangerschaft Eine stabile Erkrankung unter schwangerschaftskompatibler Medikation stellt einen der wichtigsten prädiktiven Faktoren für einen guten Schwangerschaftsverlauf dar. Die Evidenz zur Sicherheit von antirheumatischen Therapien steigt stetig. Aktuelle Empfehlungen sollten in der Beratung beachtet werden.

Starting in mid-May 2020, many US states began relaxing social distancing measures that were put in place to mitigate the spread of COVID-19. To evaluate the impact of relaxation of restrictions on COVID-19 dynamics and control, we developed a transmission dynamic model and calibrated it to US state-level COVID-19 cases and deaths. We used this model to evaluate the impact of social distancing, testing and contact tracing on the COVID-19 epidemic in each state. As of July 22, 2020, we found only three states were on track to curtail their epidemic curve. Thirty-nine states and the District of Columbia may have to double their testing and/or tracing rates and/or rolling back reopening by 25%, while eight states require an even greater measure of combined testing, tracing, and distancing. Increased testing and contact tracing capacity is paramount for mitigating the recent large-scale increases in U.S. cases and deaths.

Entzundlich rheumatische Erkrankungen betreffen haufig Frauen im gebarfahigen Alter, fur die eine Schwangerschaft nach wie vor mit erhohten Risiken einhergeht. Gleichzeitig gewinnen wir immer mehr Einblick in Risikofaktoren und Praventionsmoglichkeiten, sodass fur die meisten Frauen erfolgreiche und komplikationsarme Schwangerschaften moglich sind. Grundpfeiler sind eine Planung der Schwangerschaft unter guter Krankheitskontrolle und die Modifikation von behandelbaren Risikofaktoren. Dabei ruckt erneut Hydroxychloroquin als Substanz mit vielfaltigen positiven Wirkungen in den Fokus. Vom American College of Rheumatology (ACR) publizierte Empfehlungen geben einen praxisnahen Uberblick zur Evidenzlage. Rheumatologen konnen, basierend auf dem aktuellen Wissensstand, nachhaltig zu einem positiven Schwangerschaftsverlauf fur Mutter und Kind beitragen.

The report documents consolidated results on open science of an expert meeting in June 2022 in the succeeding order: open science focal points in June 2022, important policy initiatives in June 2022, and conclusions for the year 2022. On 16 June 2022, Path2Integrity organised a conference at the Steigenberger Hotel in Kiel involving six informed stakeholders to discuss priorities for promoting open science in Europe. The stakeholders represented research-funding and research-producing organisations with different fields of interest, specifically open science infrastructure, educational and legal best practices, national and international science agents, research ethics, and the issue of reproducibility. This gender-balanced group discussed how far Europe had come in the open science movement and how research quality, efficiency and creativity and society's trust in science could be improved. They focused on tackling the reproducibility crisis, responding to societal challenges, accessing and sharing results, and combating the knowledge gap concerning the FAIR principles. This report concisely identifies and examines the issues that have been discussed in the small circle meetup on open science., Written on behalf of the six experts attending the small circle meetup.

Background Uncontrolled inflammatory disease activity can impact pregnancy outcomes and the health of the mother and child. This retrospective claims database analysis assessed treatment patterns before, during, and after pregnancy among women with inflammatory rheumatic disease (IRD; axial spondyloarthritis [axSpA], psoriatic arthritis [PsA], and rheumatoid arthritis [RA]) or psoriasis (PSO) in Germany. Methods Data were extracted from the BARMER sickness fund (2013–2017). Pregnant women (18–45 years) with documented IRD or PSO diagnoses were compared with age-matched controls from the same database for the analysis of patient characteristics, healthcare resource utilization, and pharmacological treatment during pregnancy. Reported measures included the proportion of women with pharmacological prescriptions or hospitalization/new prescription of corticosteroids or biologics in the 180 days before pregnancy, during pregnancy, and 180 days after delivery. Pre-specified prescription categories (such as disease-specific drugs [not including biologics]) were identified by anatomical therapeutic chemical classification codes. Extrapolated values to the German statutory health insurance population are reported. Results Overall, 2702 pregnant women with IRD (axSpA: 1063; PsA: 660; RA: 979) and 6527 with PSO were identified. The proportion of women with IRD receiving prescriptions for disease-specific drugs reduced during pregnancy and remained stable after delivery (before: 15.0%; during: 9.0%; after: 9.7%). The proportion of women with PSO receiving prescriptions for disease-specific drugs was low (before: 0.6%; during: 0.3%; after: 0.1%). The proportion of women with hospitalization/new prescription of corticosteroids or biologics decreased during pregnancy, compared with pre-pregnancy, and increased after delivery in women with IRD (before: 9.0%; during: 5.1%; after: 11.1%) and PSO (before: 3.5%; during: 1.9%; after: 2.7%). Conclusions A reduction in pharmacological treatment during pregnancy was observed for women with IRD in Germany. Many women with IRD did not return to pre-pregnancy treatments after delivery, despite signs of disease exacerbation, such as hospitalization and initiation of treatment with corticosteroids/biologics, in this period. Supplementary Information The online version contains supplementary material available at 10.1007/s40744-021-00347-3.

Eine Behandlung des systemischen Lupus erythematodes (SLE) ohne dauerhafte Glukokortikoidtherapie ist fur die meisten Patienten und auch deren Arzte nicht vorstellbar. Zwar konnte die Prognose des SLE deutlich verbessert werden, das Management ist aber unverandert durch einen Mangel an wirksamen, zielgerichteten Therapien und das Fehlen evidenzbasierter Ansatze zum Einsatz der vorhandenen Wirkstoffe eingeschrankt. So gibt es fur die Glukokortikoide (GC), die bei einer Mehrzahl der Patienten kontinuierlich eingesetzt werden, keine evidenzbasierten Empfehlungen zur Einleitung, Reduktion und Beendigung in der Behandlung des SLE. Glukokortikoide sind auch heute in akuten, v. a. organ- oder lebensbedrohenden, Situationen alternativlos, allerdings wird ihre Rolle aufgrund der bekannten unerwunschten Langzeitwirkungen zunehmend kritisch gesehen. Langzeitdaten aus Kohorten belegen, dass der Einsatz von GC tatsachlich zu Morbiditat und Mortalitat beim SLE beitragt. Strategien zur Reduktion des GC-Einsatzes beim SLE sind daher dringend erforderlich und werden in diesem Beitrag vorgeschlagen.

The Physician Global Assessment International Standardisation COnsensus in Systemic Lupus Erythematosus (PISCOS) study aimed to obtain an evidence-based and expert-based consensus standardisation of the Physician Global Assessment (PGA) scoring of disease activity in systemic lupus erythematosus (SLE). An international panel of 79 SLE experts participated in a three-round Delphi consensus process, in which 41 statements related to the PGA in SLE were rated, using a 0 (strongly disagree) to 10 (strongly agree) numerical rating scale. Statements with agreement of 75% or greater were selected and further validated by the expert panel. Consensus was reached on 27 statements, grouped in 14 recommendations, for the use of the PGA in SLE, design of the PGA scale, practical considerations for PGA scoring, and the relationship between PGA values and levels of disease activity. Among these recommendations, the expert panel agreed that the PGA should consist of a 0–3 visual analogue scale for measuring disease activity in patients with SLE in the preceding month. The PGA is intended to rate the overall disease activity, taking into account the severity of active manifestations and clinical laboratory results, but excluding organ damage, serology, and subjective findings unrelated to disease activity. The PGA scale ranges from “no disease activity” (0) to the “most severe disease activity” (3) and incorporates the values 1 and 2 as inner markers to categorise disease activity as mild (≥0·5 to 1), moderate (>1 and ≤2) and severe (>2 to 3). Only experienced physicians can rate the PGA, and it should be preferably scored by the same rater at each visit. The PISCOS results will allow for increased homogeneity and reliability of PGA ratings in routine clinical practice, definitions of remission and low disease activity, and future SLE trials.

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.

ZusammenfassungDie Veröffentlichung der neuen ACR/EULAR Klassifikationskriterien für den systemischen Lupus erythematodes (SLE) und die Überarbeitung der EULAR-Empfehlungen zum Management des SLE im vergangenen Jahr beinhalten viele wichtige Neuerungen, die die Klassifikation und Versorgung von Lupus-Patienten verändern werden. Dies bezieht sich sowohl auf generelle Grundprinzipien der Versorgung sowie auf konkrete Therapieempfehlungen. So kann ein SLE erst als solcher klassifiziert werden, wenn antinukleäre Antikörper (ANA) unabhängig von Titer-Höhe und Fluoreszenzmuster nachgewiesen werden können. Sind ANA nachgewiesen, erhalten Lupus-spezifische Symptome und serologische Auffälligkeiten einen Punktwert. Ab einem Wert von 10 kann ein SLE-Patient als solcher klassifiziert werden. Im Hinblick auf die Versorgung von SLE-Patienten sollte jede Therapie ganz im Sinne eines treat-to-target Ansatzes mit dem Ziel der Remission verfolgt werden. Dies dient der Sicherung des Langzeitüberlebens, der Reduktion von Schaden sowie der Verbesserung von Lebensqualität. Erreicht werden diese Ziele durch konsequente Therapie und regelmäßige Evaluation der Krankheitsaktivität. So sollen alle Patienten bei Abwesenheit von Kontraindikationen Antimalariamittel erhalten. Bei unzureichendem Ansprechen werden Steroidpulse über wenige Tage sowie der frühzeitige Einsatz von Immunsuppressiva (IM) empfohlen. Die einzelnen IM und ihre jeweiligen Indikationen sind im Text aufgeführt. Mit dem Einbezug von Biologika, insbesondere Belimumab und Rituximab, haben außerdem nun ganz neue Substanzklassen Einzug in die Empfehlungen gefunden.

Antiphospholipid syndrome (APS) was first identified in patients with systemic lupus erythematosus (SLE) and frequent occurrence of thromboembolic complications and miscarriages accompanied by detection of anticardiolipin antibodies (aCL). When APS was also later found without an underlying SLE, the so-called primary APS was distinguished from its secondary form with SLE. Even more specific than aCL are the lupus anticoagulant (LA) and antibodies against beta‑2 glycoprotein I (aB2GP I). In recent years, it has become evident that the risk of (further) thromboembolic and obstetric complications is markedly increased if all three serological criteria of APS (aCL, aB2GP I and LA), the so-called triple positivity, are present (high-risk profile). Immunosuppression is not effective in preventing further thromboembolic complications of APS. Low-dose aspirin (LDA), heparin and vitamin K antagonists are used in primary and secondary prophylaxis. The direct oral anticoagulants have an increased risk of complications compared to these treatments and should not be used in cases of high-risk APS.

ObjectiveTo investigate outcome and course of pregnancies in women with axial spondyloarthritis (axSpA) in a pooled data analysis of pregnancy registries in rheumatology.MethodsProspectively followed women with axSpA, fulfilling ASAS classification criteria and for whom a pregnancy outcome was reported, were eligible for the analysis. Anonymised data of four registries was pooled. Rates of adverse pregnancy outcomes were calculated. Systemic inflammation, disease activity and treatment patterns with tumour necrosis factor inhibitor (TNFi) before, during and after pregnancy were analysed.ResultsIn a total of 332 pregnancies from 304 axSpA women, 98.8% of the pregnancies resulted in live birth. Mean maternal age was 31 years and disease duration 5 years. Most of these patients received pre-conception counselling (78.4%). Before pregnancy, 53% received TNFi treatment, 27.5% in first and 21.4% in third trimester. Pregnancy and neonatal outcomes were favourable with rates of 2.2% for pre-eclampsia, 4.9% for preterm birth, 3.1% for low birth weight and 9.5% for small for gestational age. Neonates were delivered by caesarean section in 27.7% of pregnancies, of which 47.4% were emergencies. Pooled mean CRP was 4 mg/L before conception peaking in the second trimester at 9.4 mg/L. Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) was below 4 at all time-points.ConclusionsPooled rates of most outcomes were better than what had been reported in the literature and within expected rates of those reported for the general population. Pre-conception counselling, planned pregnancies and a tight management in expert centres applying a tailored treatment approach may have contributed to the favourable pregnancy outcomes.

Purpose The goal of this study is to examine the chronological development of hospitalized oncology and COVID-19 patients, and compare effects on oncology sub-disciplines for pre-pandemic (2017–19) and pandemic (2020–21) years in the setting of a German university maximum care provider. Methods Data were retrospectively retrieved from the hospital performance controlling system for patient collectives with oncological main (nOnco) and COVID-19 secondary diagnosis (nCOVID-19). Data analysis is based on descriptive statistical assessment. Results The oncology patient collective (nOnco = 27,919) shows a decrease of hospitalized patients for the whole pandemic (− 4% for 2020 and − 2,5% for 2021 to 2019). The number of hospitalized COVID-19 patients increases from first to second pandemic year by + 106.71% (nCOVID-19 = 868). Maximum decline in monthly hospitalized oncology patients amounts to − 19% (May 2020) during the first and − 21% (December 2020) during the second lockdown. Relative monthly hospitalization levels of oncology patients reverted to pre-pandemic levels from February 2021 onwards. Conclusion The results confirm a decline in hospitalized oncology patients for the entire pandemic in the setting of a maximum care provider. Imposed lockdown and contact restrictions, rising COVID-19 case numbers, as well as discovery of new virus variants have a negative impact on hospitalized treated oncological patients.

ObjectiveMultiple guidelines recommend continuing hydroxychloroquine (HCQ) for systemic lupus erythematosus (lupus) during pregnancy based on observational data. The goal of this individual patient data meta-analysis was to combine multiple datasets to compare pregnancy outcomes in women with lupus on and off HCQ.MethodsEligible studies included prospectively-collected pregnancies in women with lupus. After a manuscript search, 7 datasets were obtained. Pregnancy outcomes and lupus activity were compared for pregnancies with a visit in the first trimester in women who did or did not take HCQ throughout pregnancy. Birth defects were not systematically collected. This analysis was conducted in each dataset and results were aggregated to provide a pooled odds ratio.ResultsSeven cohorts provided 938 pregnancies in 804 women. After selecting one pregnancy per patient with a 1st trimester visit, 668 pregnancies were included; 63% took HCQ throughout pregnancy. Compared to pregnancies without HCQ, those with HCQ had lower rates of highly active lupus, but did not have different rates of fetal loss, preterm birth, or preeclampsia. Among women with low lupus activity, HCQ reduced the risk for preterm delivery.ConclusionThis large study of prospectively-collected lupus pregnancies demonstrates a decrease in SLE activity among woman who continue HCQ through pregnancy and no harm to pregnancy outcomes. Like all studies of HCQ in lupus pregnancy, this study is confounded by indication and non-adherence. As this study confirms the safety of HCQ and diminished SLE activity with use, it is consistent with current recommendations to continue HCQ throughout pregnancy.

BACKGROUND: Pregnancies in women with chronic disease are on the rise. This pertains to autoimmune diseases in particular since these tend to affect women of childbearing age. The interaction between pregnancy and autoimmune disease may increase the risk of maternal, fetal, and obstetric complications; additional care may be required. METHODS: This review is based on a selective literature search in PubMed (2015–2020). RESULTS: In women with autoimmune diseases, the course of pregnancy is highly variable. Some autoimmune diseases tend to improve during pregnancy and do not to result in any serious obstetric complications. Others may worsen during pregnancy, with deterioration of the maternal condition as well as obstetric and perinatal complications. In systemic lupus erythematosus and myasthenia gravis, placental transfer of specific autoantibodies may cause fetal or neonatal disease. CONCLUSION: The care of pregnant women with chronic diseases requires collaboration between specialists of the pertinent levels of care. A stable course of disease before conception, close interdisciplinary care, and pregnancy-compatible medication contribute to a reduction in maternal and perinatal complications.

Objective. Health-Related Quality of Life (HRQoL) in adult patients with mixed connective tissue disease (MCTD) has not been described so far. Therefore, we performed an explorative study to evaluate HRQoL in MCTD patients.Methods. MCTD patients fulfilling the Kahn criteria and participating in the prospective follow-up cohort for MCTD of the Leiden University Medical Center were included; and matched to systemic sclerosis (SSc) patients based on age, sex and disease duration. Data on disease characteristics and HRQoL (SF36 and EQ-5D) were collected annually. HRQoL was compared between MCTD and SSc patients at baseline. Factors associated with HRQoL in MCTD were identified using linear regression and change in HRQoL over 3 years using linear mixed models.Results. Thirty-four MCTD patients (121 visits) and 102 SSc patients (424 visits) were included. At baseline, MCTD patients presented with interstitial lung disease, cardiac involvement, synovitis and myositis more frequently compared to SSc patients, while use of immunosuppressive medication was less frequent. In both groups, mean SF36 scores were lower than in the general Dutch population. The SF36 subscore "general health perception" was impacted most in both groups (MCTD: 38.5 [SD:7.0], SSc: 39.9 [SD:8.9]). During follow-up, SF36 scores improved in MCTD patients, while EQ5DNL remained stable. No specific characteristics were identified that associated with baseline HRQoL or change in HRQol over time.Conclusion. Like in SSc, HRQoL in MCTD is significantly impaired, especially the general health perception of patients. Evaluation in larger prospective cohorts is needed to identify characteristics that impact HRQol most.

In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.

ObjectiveIt is still a matter of debate whether low-dose acetylsalicylic acid (LDASA) should be prescribed to all patients with SLE during pregnancy. This study aimed at investigating the impact of LDASA on pregnancy outcomes in patients with SLE without history of renal involvement and without antiphospholipid antibodies (aPL).MethodsThis is a retrospective analysis of prospectively monitored pregnancies at seven rheumatology centres. Previous/current renal involvement and aPL positivity were the exclusion criteria. Adverse pregnancy outcome (APO) is the composite outcome of the study and included proteinuric pre-eclampsia, preterm delivery Results216 pregnancies in 187 patients were included; 82 pregnancies (38.0%) were exposed to LDASA treatment. No differences in terms of age at conception, disease duration, clinical manifestations, comorbidities and disease flare during pregnancy were observed between patients taking LDASA and those who did not take LDASA during pregnancy. APO was observed in 65 cases (30.1%), including 13 cases (6.1%) of pre-eclampsia. The incidence of all complications was similar in the two groups. However, it is interesting to note that pre-eclampsia had lower frequency in patients taking LDASA versus those not taking LDASA (2.4% vs 8.3%, p=0.14).ConclusionsIn pregnant patients with SLE without renal involvement and were aPL-negative, there is a low risk of severe obstetric complications, such as early pre-eclampsia. LDASA treatment does not provide a statistically significant advantage over these complications. However, a careful individual risk–benefit balance is warranted.

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.

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.

Johanna Mucke, Ralph Brinks, Rebecca Fischer-Betz, Jutta G Richter, Oliver Sander, Matthias Schneider, Gamal Chehab Policlinic and Hiller Research Unit for Rheumatology, Heinrich Heine University Duesseldorf, Duesseldorf, GermanyCorrespondence: Johanna MuckePoliclinic and Hiller Research Unit for Rheumatology, Heinrich-Heine-University, Moorenstrasse 5, Duesseldorf 40225, GermanyTel +49 211 811 7817Fax +49 211 811 9206Email Johanna.Mucke@med.uni-duesseldorf.dePurpose: Since the launch of belimumab in 2011, the BLyS antibody has been increasingly used in the therapy of systemic Lupus erythematosus (SLE). Comparative studies showed that the intravenous (i.v.) and subcutaneous (s.c.) administration forms do not differ in their efficacy. Since the approval of the s.c. therapy, many patients have been switched from i.v. to s.c. administration. The clinical course of these patients and their satisfaction regarding the drug have not yet been investigated.Methods: A total of 9 patients with SLE were switched from i.v. to s.c. belimumab between 12/2017 and 03/2018. We assessed a self-developed questionnaire on drug satisfaction, disease activity (SLEDAI-2k), serological activity (leukocytes, DNA antibodies, complement), disease damage (SLICC/ACR damage index) and functional status (health-assessment questionnaire) at switching (T0) and after 6 months (T1). Association of the questionnaires with the form of administration (i.v. vs s.c.) was analyzed for each variable separately by linear regression analyses, adjusted for age, gender and disease duration.Results: At switching, disease activity of all patients was well controlled (median SLEDAI-2k = 2 [Interquartile range 0–4]) and the patients were mainly satisfied with their therapy. No evidence for any difference in disease activity, disease damage or patient satisfaction 6 months after switching was found. In tendency, patients were more satisfied with the s.c. administration.Conclusion: The switch from i.v. to s.c. belimumab was successful in all cases and had no effect on disease activity or patient satisfaction. Despite the small sample size, s.c. belimumab seems to offer a good alternative to i.v. application.Keywords: SLE, patient satisfaction, b-cell therapy, BLyS-antibody

Die Verbesserung der gesundheitsbezogenen Lebensqualitat (HRQoL) ist ein ubergeordnetes Behandlungsziel beim systemischen Lupus erythematodes (SLE). Patientendaten mit wiederholten HRQoL-Messungen aus unserer Ambulanz wurden retrospektiv analysiert, um Faktoren zu ermitteln, die sich auf die HRQoL bei SLE auswirken. Insbesondere sollte der Zusammenhang zwischen HRQoL und Krankheitsaktivitat ermittelt werden. Outcomes Study Short Form 36 (SF-36) und Systemic Lupus Activity Measure (SLAM) sowie klinische Routinedaten von 169 Patienten (83 % weiblich, Alter 40,3 ± 13 Jahre [MW ± SD], Krankheitsdauer 9,4 ± 7 Jahre) uber 7,1 ± 4,2 Jahre wurden mittels gemischt linearer Modelle analysiert, um Einflussfaktoren auf die physische (PCS) und mentale (MCS) Lebensqualitat gemas SF-36 zu ermitteln. Der Anteil der HRQoL, die sich durch die Variablen erklaren lies, wurde mittels marginal R2 (mR2) und conditional R2 (cR2) abgeschatzt. Zu Studienbeginn zeigen SLE-Patienten eine reduzierte HRQoL in allen Kategorien des SF-36 inklusive der PCS und MCS mit Ausnahme der Vitalitat. Ein hoher PCS war uber die Zeit signifikant assoziiert zu begleitender Einnahme von Antimalariamitteln, keinen Glukokortikoiden, geringerer Fatigue, geringerer Krankheitsaktivitat sowie zu den Ausgangsparametern jungeres Alter und hoher PCS (mR2 54,7 %, cR2 59,9 %). Ein hoher MCS war assoziiert zu begleitender Einnahme von Glukokortikoiden und einem hoheren Ausgangs-MCS (mR2 21,7 %, cR2 25,1 %). Antimalariamittel und keine Glukokortikoide in der Medikation sowie eine niedrige Krankheitsaktivitat sind modifizierbare Faktoren, die mit einer besseren physischen Lebensqualitat assoziiert sind. Die mentale Komponente des HRQoL wird schlecht durch konventionelle klinische Parameter widergespiegelt und zeigte keine Assoziationen zu Parametern der Krankheitsaktivitat in der aktuellen Kohorte.

Solange wir Erkrankungen wie den systemischen Lupus erythematodes (SLE) nicht heilen konnen, muss es unser Ziel sein, dass Menschen mit SLE ein weitgehend normales Leben fuhren durfen. Es sollte nicht von ihrer Erkrankung dominiert werden. Ziel ist v. a. die Sekundarpravention, d. h. die Verhinderung von Schaden. Im Jahr 2020 wollen wir dafur einen besseren Beitrag leisten. Fit sein fur 2020 bedeutet daher fur diesen Artikel zum SLE, die Grundkonzepte der Versorgung von Patienten mit SLE regelhaft anzuwenden. Der Fokus gilt dabei v. a. den neuen Konzepten, die sich aus den neuen Klassifikationskriterien ergeben, der optimierten Grundversorgung (BASIC), der komplexen Kommunikation mit den Betroffenen, den neuen EULAR(European League Against Rheumatism)-Empfehlungen zum Monitoring von SLE, der Integration von Biologika in das Behandlungsregime, der zielgerichteten Therapie (T2T) und damit den Kriterien „low disease activity“ und Remission, der Frage nach neuen Versorgungsstrukturen fur seltene und komplexe Systemerkrankungen und abschliesend dem Ausblick auf zukunftige Behandlungskonzepte, deren Basis wir alle in den kommenden Jahren erschaffen werden. Wenn wir die aktuellen Moglichkeiten konsequent anwenden, konnen wir auch ohne neu zugelassene Medikamente die Lebenssituation und -qualitat der Betroffenen weiter verbessern.

OBJECTIVE To investigate the role of epitope spreading in established systemic lupus erythematosus (SLE). METHODS IgG autoantibody reactivity with 398 distinct recombinant proteins was measured over a period of 6 years in 69 SLE patients and compared to that in 45 controls. Changes in mean fluorescence intensity (MFI), number of autoantibodies to distinct antigens, and reactivity with distinct clones of established antigenic targets (e.g., U1 RNP, Sm, and ribosomal P) representing epitope fine mapping were assessed. Linear mixed modeling, adjusted with Bonferroni correction for age and sex, was applied. RESULTS The total number of autoantibodies, mean MFI, and number of autoantibodies in epitope fine mapping were higher in SLE patients compared to controls (P < 0.0001). The total number of antibodies to distinct autoantigens remained stable over time, while the mean MFI decreased over time in SLE (P < 0.021). SLE patients showed variable recognition of epitopes in fine mapping over time. In particular, in SLE patients, more clones of the U1 RNP complex were recognized at the time of new organ involvement (+0.65) (P = 0.007). Mean MFI was higher in patients with lupus nephritis (P = 0.047). The time-averaged MFIs of 22 individual autoantibodies (including double-stranded DNA [dsDNA]) were higher, after Bonferroni correction, in SLE (P < 0.0001). The MFIs of dsDNA and histone cluster 2 H3c were associated with scores on the Systemic Lupus Activity Measure (P < 0.0001). CONCLUSION Longitudinal surveillance of the IgG autoantibody repertoire in established SLE reveals evidence of sustained breadth of autoantibody repertoire without significant expansion. Associations of disease activity with dsDNA and with histone H3 autoantibodies were confirmed.

Inflammatory rheumatic diseases often affect women of childbearing age, for whom pregnancy is still associated with increased risks. At the same time, we are gaining more and more insights into risk factors and preventive strategies, enabling almost all women to have successful pregnancies with few adverse outcomes. The cornerstones are planning of the pregnancy under effective disease control and the modification of individual risk factors. Hydroxychloroquine is once again coming into focus as a compound with multiple positive effects. Recommendations published by the American College of Rheumatology (ACR) provide a practical overview of the evidence. Based on the current state of knowledge, rheumatologists can make a lasting contribution to a positive pregnancy outcome for mother and child.Entzündlich rheumatische Erkrankungen betreffen häufig Frauen im gebärfähigen Alter, für die eine Schwangerschaft nach wie vor mit erhöhten Risiken einhergeht. Gleichzeitig gewinnen wir immer mehr Einblick in Risikofaktoren und Präventionsmöglichkeiten, sodass für die meisten Frauen erfolgreiche und komplikationsarme Schwangerschaften möglich sind. Grundpfeiler sind eine Planung der Schwangerschaft unter guter Krankheitskontrolle und die Modifikation von behandelbaren Risikofaktoren. Dabei rückt erneut Hydroxychloroquin als Substanz mit vielfältigen positiven Wirkungen in den Fokus. Vom American College of Rheumatology (ACR) publizierte Empfehlungen geben einen praxisnahen Überblick zur Evidenzlage. Rheumatologen können, basierend auf dem aktuellen Wissensstand, nachhaltig zu einem positiven Schwangerschaftsverlauf für Mutter und Kind beitragen.