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IntroductionSLE disease activity score (SLE-DAS) is a novel, rapid, continuous and comprehensive score that overcomes the drawbacks of SLEDAI-2 K. Low lupus disease activity state (LLDAS) has been targeted as an endpoint in many clinical trials and as a favourable outcome in clinical practice. Therefore, our objective in the current study is to evaluate the validity of SLE-DAS for defining LLDAS as an objective in the treat-to-target strategy.MethodsA cross-sectional study was carried out on 117 SLE patients who were diagnosed according to Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE. Patients were evaluated for disease activity by both SLEDAI-2 K and SLE-DAS. Additionally, patients were divided according to the SLEDAI-2 K-derived LLDAS definition into two groups: low disease activity (LDA) group and high disease activity (HDA) group. The validity of SLE-DAS for the definition of LLDAS was evaluated in comparison to SLEDAI-2 K.ResultsSLE-DAS shows highly significant positive correlation (r = 0.743, p < 0.001) with SLEDAI-2 K. The ROC curve revealed that SLE-DAS is valid for the assessment of activity with the best detection of LLDAS was at 6.62 with 95.5% sensitivity, 79.3% specificity and 89.6% accuracy. Moreover, it had a good agreement with the SLEDAI-2 K-derived definition of LLDAS (k = 0.765, p < 0.001).ConclusionSLE-DAS is a valid score for the definition of LLDAS which can be used in the clinical practice as a simple and precise standalone criterion.Key Points• Correlation between SLEDAI-2K and SLE-DAS revealed a high significance.• Identify SLE-DAS cut-off 6.62 for the definition of LLDAS.• There is a good agreement between SLEDAI-2K-derived definition of LLDAS and SLE-DAS definition.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by high hetero­geneity of clinical manifestations and an uncertain prognosis. Although the mortality rate due to SLE has decreased significantly in recent decades, there is still a need to find good tools to measure disease activity for early detection of exacerbations and treatment planning. Over the decades, more than a dozen disease activity scales/indicators have been developed, with the SLE Disease Activity Index (SLEDAI) being the most popular. More recently, the new SLE Disease Activity Score (SLE-DAS) has been introduced. This paper compares the two methods of assessing SLE activity, and presents the relevance of these scales in pregnant SLE patients and their use in formulating definitions of remission and low disease activity. The results show that the SLEDAI and the SLE-DAS are of comparable value in assessing SLE activity and complement each other.

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects multiple organ systems and manifests in a relapsing–remitting pattern. Consequently, it is paramount for rheumatologists to assess disease activity, identify flare-ups, and establish treatment goals for patients with SLE. In 2019, the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) was introduced as a novel tool for measuring disease activity. This tool refines the parameters of the established SLE Disease Activity Index 2000 (SLEDAI-2K) to enhance the assessment process. This review aims to provide an introduction to the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) and summarizes research on its development, its comparison with existing disease activity measures, and its performance in clinical settings. Literature searches on PubMed using the keyword “SLE-DAS” were conducted, covering publications from March 2019 to September 2023. Studies that compared SLE-DAS with other SLE disease activity measurement tools were reviewed. Findings indicated that SLE-DAS consistently performs on par with, and sometimes better than, traditional measures in assessing clinically meaningful changes, patient improvement, disease activity, health-related quality of life, hospitalization rates, and disease flare-ups. The association between SLE-DAS and mortality rates among patients with SLE, however, remains to be further explored. Although SLE-DAS is a promising and potentially effective tool for measuring SLE disease activity, additional research is needed to confirm its effectiveness and broaden its clinical use.

Introduction: Systemic Lupus Erythematosus (SLE) mainly occurs during childbearing age. Remission or low disease activity state (LDAS) before conception are recommended by experts to achieve a favourable lupus pregnancy outcome but little is known on the best way to evaluate remission or activity status during pregnancy. Objectives: We tested SLE-disease activity score (SLE-DAS) in the first trimester as predictor of maternal flares and obstetrical complications in 2nd and 3rd trimester in a cohort of SLE pregnant women. Patients and Methods: Inclusion criteria were: 1) women ≥ 18 years; 2) affected with SLE (SLICC 2012); 3) enrolled in two referral centers (Italy and France) 4) with an ongoing singleton pregnancy at 12 weeks (only one pregnancy per patient). Disease activity was assessed at first trimester of pregnancy, using SLE-pregnancy disease activity index (SLEPDAI) and retrospectively applying SLE-DAS. Maternal lupus flares at 2nd and 3rd trimester were defined by the SELENA-SLEDAI Flare Index (SFI). Adverse pregnancy outcome (APO) included: fetal and neonatal death, placental insufficiency with premature delivery <37 weeks, and small for gestational age (SGA) (≤3rd percentile). Results: We included 158 pregnant patients affected with SLE. At first trimester the median SLEPDAI (IQR) was 2 (0–4) and the median SLE-DAS (IQR) 1.32 (0.37–2.08). At least one flare occurred in 25 (15.8%) women during the 2nd and 3rd trimester. APO occurred in 19 (12.0%) patients. A significant correlation between SLE-DAS and SLEPDAI was found in this cohort (Spearman’s ρ = 0.97, Figure 1 ). At multivariate analysis, both SLE-DAS and SLEPDAI predicted maternal flares (adjOR = 1.2; 95% CI = 1.0–1.3, p = 0.02; adjOR 1.3, 95% CI = 1.1–1.6 per unit increase, p = 0.01, respectively). SLE-DAS and SLEPDAI were associated with APO at univariate analysis ( p = 0.02). Conclusions: SLE-DAS was highly correlated with SLEPDAI and its use in the first trimester predicted maternal flares in the 2nd and 3rd trimester, making SLE-DAS a reliable instrument to measure SLE activity during pregnancy.

Background Systemic lupus erythematosus (SLE) affects reproductive aged women. Women with SLE can flare during pregnancy, in particular those who have active disease at conception or prior history of renal disease. These flares can lead to increased adverse pregnancy outcomes including fetal loss, preeclampsia, preterm birth, and small for gestational age infants. Objective To evaluate SLE Disease Activity Score (SLE-DAS) in the first trimester as a predictor of maternal flares in 2nd and 3rd trimester and adverse pregnancy outcome (APO) in SLE pregnant women. Patients and methods This cohort study was done on 50 SLE patients. Disease activity and maternal flares were assessed by applying SLE-DAS at first, second, and third trimester, subdivided into mild/moderate and severe. Follow-up: Visits were scheduled every trimester and postpartum to detect fetal outcomes. Results Regarding maternal outcomes, 22% of patients developed disease flare-up during pregnancy. We found significantly younger age at pregnancy and higher disease activity in the first trimester among flare group in 2nd and 3rd trimester than non-flare group ( p  = 0.023, 0.000, respectively). Twenty-four percent of patients had at least one APO, 41 patients (82.0%) had live birth, 36% developed APO, 16% of them were preterm birth due to placental insufficiency, 16% developed IUFD ≥ 12 weeks, and 4% had small for gestational age infants. Conclusion APO in lupus patients was significantly associated with higher disease activity in the first trimester; so proper control of disease activity improves pregnancy outcomes.

Abstract Capturing disease activity in SLE remains challenging. The binary nature of global score indices such as the SLEDAI 2000 (SLEDAI-2K) poses limitations, while the complexity of the BILAG-2004 index requires training and more time investment. Recent efforts to improve SLE activity indices include the SLE Disease Activity Score (SLE-DAS) and Easy-BILAG system. This review analyses the main indices used to assess SLE activity, examines their progressive refinements, evaluates their advantages and limitations and aims to identify the optimal index. The SLE-DAS offers greater sensitivity than the SLEDAI-2K and the Easy-BILAG simplifies scoring while maintaining the comprehensiveness of the BILAG-2004. Composite indices like the SLE Responder Index and BILAG-based Composite Lupus Assessment integrate the SLEDAI-2K and BILAG-2004 but are mainly used in clinical trials due to their complexity. This review emphasizes the importance of balancing sensitivity, specificity, simplicity and comprehensiveness in lupus activity measurement. The search for the optimal index remains ongoing. Lay Summary What does this mean for patients? Capturing disease activity accurately and completely in lupus patients is difficult because the disease causes many clinical problems. Some problems may be hard to distinguish from other factors, including concomitant diseases and drug side effects. Global score systems offer a simplistic way to capture disease activity (‘awarding’ points if a feature is present). The best-known example of this approach, the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) system, has been criticized, as it misses some features altogether (e.g. gastrointestinal involvement) and the logic for the ‘weighting’ of some features (e.g. a lupus headache—considered rare—gets 8 points) is hard to justify. However, it is very easy to calculate. In contrast, the British Isles Lupus Assessment Group (BILAG) system is more comprehensive and (unlike the SLEDAI) distinguishes partial improvement in features from those that remain unchanged and those that have gotten worse. However, it takes longer to complete. In this review we compared some ‘new kids on the block’, focusing in particular on the SLE Disease Activity Score system, which is more comprehensive than the SLEDAI index, and the Easy-BILAG, which uses a colour-coding approach to help get to the final score much faster. Time will tell if either, or both, of these new instruments will become more widely used.

Zusammenfassung Hintergrund Der systemische Lupus erythematodes (SLE) ist eine klinisch heterogen verlaufende Autoimmunerkrankung, die mit hohem Leid für die Betroffenen sowie hohen sozioökonomischen Kosten verbunden ist. Eine frühe Diagnosestellung und eine adäquate medizinische Versorgung sind essenziell für einen milden Krankheitsverlauf. Es fehlen jedoch aktuelle Zahlen und Daten über die Versorgungssituation der Erkrankten in der Fläche. Methodik Es wurden insgesamt 1546 Hausärzte, Rheumatologen, Neurologen, Nephrologen und Dermatologen in Rheinland-Pfalz und dem Saarland per Fax oder Mail mithilfe eines Fragebogens bezüglich Epidemiologie, Symptomatik, Therapie und Therapieerfolg befragt. Zusätzlich gab es die Möglichkeit, Verbesserungsvorschläge zu unterbreiten. Ergebnisse Fünf von sechs der rückgemeldeten 635 SLE-Patienten sind weiblich. Die häufigsten Hauptsymptome waren Arthralgien, Fatigue, Myalgien und Hautveränderungen. Von den Patienten erhielten 68 % Antimalariamittel (AMM), während 46 % mit Glukokortikoiden (GC) und 50 % mit einem Immunsuppressivum (IS), v. a. Methotrexat (MTX), behandelt wurden. An Komorbiditäten litten die Patienten vor allem unter kardiovaskulären Erkrankungen, dem Fibromyalgiesyndrom und Depressionen. Rheumatologen beschrieben zudem häufig Anämien, Diabetes mellitus und Osteoporose. Diskussion Verglichen mit den Empfehlungen der Leitlinien fiel insbesondere bei nicht rheumatologisch betreuten Patienten die geringe Quote an AMM in der Therapie auf (35 % im Mittel im Vergleich zu 81 % bei Rheumatologen). Auch (dauerhaft) hohe GC-Dosen entsprechen nicht den Empfehlungen der Literatur. Im Freitextfeld wurden vor allem mehr niedergelassene Rheumatologen und eine schnellere Terminvergabe sowie eine bessere Kommunikation und Vernetzung gewünscht. Zudem wurde häufig der Wunsch nach mehr Fortbildung und Aufklärung geäußert.

Zusammenfassung Eine 42-jährige Patientin wird wegen akuter neurologischer Symptomatik (Dysarthrie, Desorientierung) stationär eingewiesen. Nach Ausschluss einer zerebralen Ischämie und Blutung wird eine Autoimmunenzephalitis diagnostiziert. Kurz zuvor war ambulant der Verdacht auf das Vorliegen eines SLE geäußert worden. Die Patientin wies eine hochentzündliche Laborkonstellation auf und entwickelte innerhalb von Tagen eine schwere Panzytopenie. Bei Vorliegen aller Diagnosekriterien wurde eine sekundäre hämophagozytische Lymphohistiozytose (sHLH) diagnostiziert und im Verlauf der zugrunde liegende SLE u. a. mittels Nierenbiopsie gesichert. Die immunsuppressive Therapie mit Etoposid und hoch dosiertem Dexamethason analog dem HLH-94-Protokoll zeigte nur temporären Erfolg. Nach drei Wochen Behandlung mit protokollgerechter Dosisreduktion musste unter laufender Therapie eine erneute Exazerbation der Symptomatik festgestellt werden. Eine erneute Dosiseskalation der eingesetzten Medikamente führte nicht zur Symptomkontrolle. Erst der Einsatz von Cyclosporin A in Kombination mit Mycophenolatmofetil (MMF) und Dexamethason konnte die Entzündungsaktivität nachhaltig kontrollieren. Nach Stabilisierung des Zustands der Patientin war eine ambulante Weiterbetreuung möglich.

Systemic Lupus Erythematosus provides a practical approach to the assessment and management of patients with this complex, multisystem autoimmune disease to improve the diagnosis and treatment of the disease and its complications.