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BackgroundSerologically active clinically quiescent (SACQ) is a clinical state of systemic lupus erythematosus (SLE) characterized by high levels of serologic markers without clinical activity. The outcome and treatment strategy after SACQ achievement remains unclear. After achieving the treatment goal, maintaining low-dose glucocorticoids has always been both a blessing and a curse [1,2]. Whether glucocorticoids discontinuation is a desirable goal in patients achieved SACQ was controversial.ObjectivesIn this multi-center prospective study, we aimed to identify the risk of flares, organ damage accumulation, and the low-dose glucocorticoids discontinuation feasibility of SLE patients who achieved the clinical state as SACQ.MethodsThis observational study was conducted based on data from the Chinese SLE treatment and research (CSTAR) registry. Demographic characteristics, autoantibody profiles, clinical manifestations, disease activity status, organ damage, and treatment profile were collected at recruitment and during follow-up. SACQ was defined as at least a 6-months period with persistent serologic activity (positive anti-dsDNA antibody, and/or hypocomplementemia), and without clinical activity. Serologically quiescent clinically quiescent (SQCQ) was defined as at least a 6-months period without serologic or clinical activity. The flare was measured according to the SELENA-SLEDAI flare index (SFI). Organ damage is principally assessed using the SLICC damage index (SDI).ResultsOf 4107 SLE patients, 1889 reached the clinical quiescent stage (990 achieved the clinical state as SACQ, and 899 patients achieved SQCQ). Among 990 SACQ patients, 364 (36.7%) underwent flares, 163(16.5%) showed organ damage, 47 (4.7%) developed renal damage, and 21 (2.1%) died during a mean follow-up of 7.30 years. Compared with patients who achieved SQCQ, SACQ patients were at a higher risk of flares (HR=1.47, 95% CI 1.25-1.73，p<0.001) and renal damage accumulation (HR=2.02, 95% CI 1.23-3.33, p=0.004). Furthermore, 224 (22.6%) SACQ patients withdraw low-dose glucocorticoids and 125 (55.8%) of them did not flare during follow-up. Low-dose glucocorticoids discontinuation was a favorable factor of long-term survival (HR=0.22, 85% CI, 0.05-0.96, P=0.044). As shown in the Figure 1, withdrawing glucocorticoids can reduce organ damage (p=0.0075), especially renal damage accumulation (p=0.045), even experience flares after discontinuation.(A) (B)Figure 1.Cumulative probability of new-onset organ damage (A) and renal damage (B) in SACQ patients maintain glucocorticoids, withdraw glucocorticoids but flares, and successful withdraw glucocorticoids.ConclusionOur results suggest that low-dose glucocorticoids withdrawal under tight survallance could be considered after achieving the clinical state as SACQ to prevent the accrual of renal damage.References[1]Apostolopoulos D, et al. The Lancet Rheumatology 2020;2(1):e24-e30.[2]Mathian A, et al. Ann Rheum Dis 2020;79(3):339-46.AcknowledgementsWe thank CSTAR co-authors as following for assistance with cases collections.Disclosure of InterestsNone Declared.

Background The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. Methods Various specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment. Results Of the 334 patients listed, 205 were male and median age at diagnosis was 65 years. The mean number of organs involved was 3.2 at diagnosis. The most frequently affected organs were the salivary glands, followed by the lacrimal glands, lymph nodes, pancreas, retroperitoneum/periaorta, kidneys, and lungs. The mean serum level of IgG4 was 755 mg/dl, and more than 95% of patients had elevated serum IgG4 levels. The median serum level of C-reactive protein (CRP) was 0.1 mg/dl and the level was less than 1 mg/dl in 90% of patients. A total of 34.7% of patients had low serum levels of C3. Serum levels of C3 and non-IgG4 IgG, calculated as the total IgG minus IgG4, showed an inverse correlation in patients with kidney lesions, while serum IgG4 levels were not correlated with serum C3 levels. Corticosteroid was administered in 78.0% of patients, and was effective in all. Conclusions The serum CRP level is generally low and the serum IgG4 level is elevated in most Japanese IgG4-RD patients, in contrast to western patients. These original findings suggest that these two parameters in IgG4-RD differ in some interesting ways from those hitherto reported in western populations. Additional studies, especially international comparative ones, are needed to elucidate the extent and significance of these differences between populations. Attention will also have to be paid to whether the existence of such differences requires consideration when devising international classification criteria.

BackgroundSjögren’s syndrome (SS) can occur alone or in combination with other autoimmune diseases. Systemic lupus erythematosus (SLE) is the most common autoimmune disease associated with SS. The prognosis of SS is generally better than that of SLE. However, the onset of SLE in these patients may be one of the factors that increase mortality.ObjectivesThis study determined the impact of demographic factors, clinical manifestations, disease activity, and serological tests at baseline on future SLE development in Sjögren’s syndrome (SS) patients.MethodsThis retrospective study assessed 1,082 SS patients without other autoimmune diseases at baseline who visited our hospital between January 2012 and March 2021. We analyzed demographic features, extra-glandular manifestations (EGMs), clinical indices, and laboratory values at baseline between the two groups divided per future SLE development (SS/SLE group vs. SS group). The probability and predictors of SLE development in SS patients were estimated using the Kaplan–Meier method and Cox proportional hazards models.ResultsThe median follow-up duration was 1083.5 days. Forty-nine patients (4.5%) developed SLE that met the 2012 Systemic Lupus International Collaborating Clinics or 2019 EULAR/ACR classification criteria. The baseline EULAR SS disease activity index (ESSDAI) score was significantly higher in the SS/SLE group (p<0.001). The SS/SLE group had more lymphadenopathy and renal involvement (p=0.015 and p=0.017, respectively). Shorter SS disease duration (<3 years) (hazard ratio [HR]=2.61, p=0.012), high ESSDAI (HR=3.04, p=0.024), leukopenia (HR=2.20, p=0.017), hypocomplementemia (HR=17.40, p<0.0001), and positive for anti-dsDNA (HR=19.93, p<0.0001), anti-ribonucleoprotein (RNP) (HR=2.96, p=0.025), and anti-ribosomal P (HR=2.74, p=0.048) at baseline were SLE development predictors in SS patients.ConclusionShorter disease duration and higher disease activity of SS at baseline may be risk factors for future SLE development. Serologic predictors of SLE development are hypocomplementemia, leukopenia, and positivity for anti-dsDNA, anti-RNP, and anti-ribosomal P antibodies. If the above factors are observed, close monitoring will be necessary during the follow-up period, considering the possibility of future SLE development.References[1]Lazarus MN and Isenberg DA. Development of additional autoimmune diseases in a population of patients with primary Sjogren’s syndrome. Ann Rheum Dis. 2005; 64: 1062-4.[2]Pasoto SG, Adriano de Oliveira Martins V and Bonfa E. Sjogren’s syndrome and systemic lupus erythematosus: links and risks. Open Access Rheumatol. 2019; 11: 33-45Figure 1.Comparison of disease activity (by ESSDAI score) and extraglandular manifestations (EGMs) at baseline between SS/SLE group and SS group.AcknowledgementsNo acknowledgements to declare.Disclosure of InterestsNone Declared.

BackgroundSome studies demonstrated that withdrawal of low-dose glucocorticoids in clinically quiescent systemic lupus erythematosus (SLE) patients increased the risk of flare [1]. An international survey of 130 clinicians showed that persistent abnormal serology led to a reluctance to reduce or discontinue medications [2].ObjectivesTo assess the risk of flare and damage accrual after tapering glucocorticoids in serologically active clinically quiescent (SACQ) patients with SLE. Association of other medications with flare in SACQ patients was also analyzed.MethodsWe used data from the Asia Pacific Lupus Collaboration cohort, prospectively collected from SLE patients (ACR/SLICC criteria) observed for at least 2 visits between 2013 and 2020. Disease activity and medication details were captured at enrolment and at routine visits. SACQ was defined at any visit as the state with serological activity (increased anti-dsDNA or hypocomplementemia) but without clinical activity as measured by SLEDAI-2K. Patients treated with 0 to 7.5 mg/day of prednisolone at a SACQ visit were analyzed after stratification according to the initial dosages of prednisolone. Cox proportional hazard models were used to assess the time-dependent relationship between decreasing prednisolone in SACQ patients and disease flares captured with the SELENA flare index at each subsequent visit, as well as subsequent damage accrual (≥1-point increase in SLICC/ACR damage index [SDI]). Each patient was observed for up to 2 years or until each outcome event occurred.ResultsFrom a total of 4,106 patients, 1,850 patients with SACQ and 8,905 visits were analyzed: 742, 271, and 180 patients experienced overall flare, severe flare, and increase in SDI, respectively. Tapering prednisolone was not associated with subsequent overall or severe flare: Each unit decrease in prednisolone dosage (1 mg/day) resulted in adjusted HRs 1.02 (95%CI, 0.99–1.05) and 0.98 (95%CI, 0.96–1.00) for overall and severe flare, respectively, in the group with initial prednisolone dosages of 0–7.5 mg/day. However, among SACQ patients, antimalarial use was significantly associated with reduced overall and severe flare in the groups with initial prednisolone of 0–7.5 or 0–5 mg/day. In addition, immunosuppressive use was significantly associated with reduced severe flare but not overall flare in these groups. Decreasing the dosage of prednisolone was significantly negatively associated with damage accrual in the groups with initial prednisolone dosages of 0–7.5 mg/day (adjusted HR [95%CI], 0.97 [0.96–0.99]) and 5–7.5 mg/day (adjusted HR [95%CI], 0.96 [0.94–0.99]) but not 0–5 mg/day (adjusted HR [95%CI], 0.98 [0.95–1.01]).Table 1.Summary results of association between decreasing the prednisolone dosages and disease flares or damage accrual in SLE patients with SACQInitial prednisolone dosage (mg/day)Overall disease flareSevere disease flareIncrease in SDI0 ≤ prednisolone ≤7.51.02 (0.99–1.05),p = 0.270.98 (0.96–1.00),p = 0.110.97 (0.96–0.99),p < 0.010 ≤ prednisolone ≤51.02 (0.98–1.06),p = 0.410.98 (0.96–1.01),p = 0.280.98 (0.95–1.01),p = 0.145 < prednisolone ≤7.51.01 (0.96–1.06),p = 0.840.98 (0.92–1.03),p = 0.410.97 (0.96–0.99),p < 0.01* HRs (95% CIs) per unit decrease in prednisolone dosages (1 mg/day) were calculated using Cox proportional hazard models and adjusted by initial prednisolone dosage, antimalarial, immunosuppressive, disease duration, SLEDAI-2K, age at visit, gender, and ethnicity.ConclusionTapering prednisolone was not significantly associated with subsequent flare in SLE patients who were SACQ. Antimalarial and immunosuppressive use were associated with reduced risk of flares in SACQ patients. Tapering prednisolone was associated with reduced risk of damage accrual in SACQ patients treated with more than 5 mg/day of prednisolone. These findings suggest glucocorticoid tapering is safe and protective in SLE patients in SACQ.References[1]Rheumatology (Oxford). 2021;60:5517[2]Lupus Sci Med. 2017;4:e000173AcknowledgementsWe acknowledge the unrestricted project grants received from AstraZeneca, BMS, Eli Lilly, GSK, Janssen, Merck Serono, and UCB to support data collection and project management contributing to this work.Disclosure of InterestsYasuhiro Katsumata Speakers bureau: GlaxoSmithKline K.K., AstraZeneca K.K., Sanofi K.K., Pfizer Japan Inc., Janssen Pharmaceutical K.K., Chugai Pharmaceutical Co., Ltd.Asahi Kasei Pharma, Astellas Pharma Inc., Mitsubishi Tanabe Pharma Corporation, Eisuke Inoue Speakers bureau: Bristol-Myers Squibb K.K., Eisai Co., Ltd., Consultant of: Nippontect Systems co., Ltd., Masayoshi Harigai Speakers bureau: AbbVie Japan GK, Ayumi Pharmaceutical Co., Boehringer Ingelheim Japan, Inc.,Bristol Myers Squibb Co., Ltd., Chugai Pharmaceutical Co., Ltd., Eisai Co., Ltd., Eli Lilly Japan K.K., GlaxoSmithKline K.K., Kissei Pharmaceutical Co., Ltd., Pfizer Japan Inc., Takeda Pharmaceutical Co., Ltd., Teijin Pharma Ltd., Consultant of: AbbVie, Boehringer-ingelheim, Bristol Myers Squibb Co., Kissei Pharmaceutical Co.,Ltd. Teijin Pharma, Grant/research support from: AbbVie Japan GK, Asahi Kasei Corp., Astellas Pharma Inc., Ayumi Pharmaceutical Co., Bristol Myers Squibb Co., Ltd., Chugai Pharmaceutical Co., Daiichi-Sankyo, Inc.,Eisai Co., Ltd., Kissei.Pharmaceutical Co., Ltd., Mitsubishi Tanabe Pharma Co., Nippon Kayaku Co., Ltd., Sekiui Medical, Shionogi & Co., Ltd., Taisho Pharmaceutical Co., Ltd., Takeda Pharmaceutical Co., Ltd., Teijin.Pharma Ltd., Rangi Kandane-Rathnayake: None declared, Worawit Louthrenoo: None declared, Alberta Hoi Speakers bureau: UCB, Janssen, Sandoz, Eli Lilly, Consultant of: Abbvie, GSK, Grant/research support from: AstraZeneca, GSK, BMS, Janssen, Merck Serono, Vera Golder: None declared, C.S. Lau Speakers bureau: AstraZeneca UK Ltd., Consultant of: AstraZeneca Pharmaceuticals LP, Jiacai Cho: None declared, Aisha Lateef: None declared, Yi-Hsing Chen: None declared, Shue Fen Luo: None declared, Yeong-Jian Jan Wu: None declared, Laniyati Hamijoyo: None declared, Zhanguo Li Consultant of: Pfizer, Roche, Janssen, Abbott, AbbVie, Bristol Myers Squibb, MSD, Celgene, Eli Lilly, GSK, Novartis, UCB Pharma, Grant/research support from: Pfizer, Roche, Janssen, Abbott, AbbVie, Bristol Myers Squibb, MSD, Celgene, Eli Lilly, GSK, Novartis, UCB Pharma, Sargunan Sockalingam Consultant of: Pfizer, AstraZeneca, ZP Therapeutics, Grant/research support from: Pfizer, AstraZeneca, ZP Therapeutics, Sandra Navarra Consultant of: Biogen, Boehringer Ingelheim, Astra Zeneca, Grant/research support from: Jannsen, Novartis, Pfizer, Glaxo Smith Kline, Leonid Zamora: None declared, Yanjie Hao: None declared, Zhuoli Zhang: None declared, Madelynn Chan: None declared, Shereen Oon: None declared, Kristine Ng Consultant of: AbbVie, Jun Kikuchi: None declared, Tsutomu Takeuchi Consultant of: AbbVie, Chugai, Mitsubishi-Tanabe, Grant/research support from: AbbVie, Mitsubishi-Tanabe, Eli Lilly Japan, Fiona Goldblatt: None declared, Sean O’Neill: None declared, Nicola Tugnet: None declared, Annie Law: None declared, Sang-Cheol Bae: None declared, Yoshiya Tanaka Speakers bureau: Behringer-Ingelheim, Eli Lilly, Abbvie, Gilead, AstraZeneca, Bristol-Myers, Chugai, Daiichi-Sankyo, Eisai, Pfizer, Mitsubishi-Tanabe, GlaxoSmithKline, Grant/research support from: Asahi-Kasei, Abbvie, Chugai, Eisai, Takeda, Daiichi-Sankyo, Behringer-Ingelheim, Naoaki Ohkubo: None declared, Sunil Kumar: None declared, Mandana Nikpour Speakers bureau: Actelion, GSK, Janssen, Pfizer, UCB, Paid instructor for: UCB, Consultant of: Boehringer Ingelheim, Certa Therapeutics, Eli.Lilly, GSK, Janssen, Pfizer, UCB, Grant/research support from: Actelion, Astra Zeneca, BMS, GSK, Janssen, UCB, Eric F. Morand Speakers bureau: AstraZeneca, EMD Serono, Gilead, Consultant of: AstraZeneca, BristolMyersSquibb, Biogen, Eli Lilly, EMD Serono, Novartis, Grant/research support from: AbbVie, Amgen, AstraZeneca, BristolMyersSquibb, Biogen, Eli Lilly, EMD Serono, Genentech, GSK, Janssen, UCB.

BackgroundThe Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) has been recently developed and validated, providing improved accuracy and sensitivity for changes in SLE disease activity in comparison to SLE Disease Activity Index 2000 (SLEDAI-2K) [1]. New recommendations to standardize the Physician Global Assessment (PGA) scoring may improve its reliability [2].ObjectivesTo assess the intra- and interrater reliability of SLE-DAS, SLEDAI-2K and PGA for measuring SLE disease activity.MethodsA set of 24 clinical vignettes were abstracted, each from a real clinical visit of patients followed at an academic lupus clinic. These vignettes were selected to include a wide spectrum of SLE manifestations, organ-system involvements, and global severity of disease activity. Abstracted data were presented in a standardized format, including demographic, past medical history, current clinical picture and treatment, laboratory, and other workup assessments. A group of 19 raters were recruited as a random multicenter sample of Rheumatologists. All raters completed a preliminary training session on scoring rules for SLE-DAS, SLEDAI-2K and PGA. Each rater scored each clinical vignette with SLE-DAS, SLEDAI-2K, and PGA through an online survey. The scoring was repeated in a second round 7-14 days after the first one. The clinical vignettes were randomly ordered for each round. Inter and intra-rater reliability of each instrument was estimated using the intraclass correlation coefficient (ICC) with 95% confidence intervals (95%CI), based on single-measurement, absolute agreement, with a two-way random effect or two-way mixed-effects model, respectively.ResultsThe 19 raters included 8 rheumatologists and 11 rheumatology trainees from 11 hospitals, with a mean of 12.1±7.1 and 3.6±0.5 years of rheumatology practice, respectively, and 78.9% of the participants assess ≤5 SLE patients per week in their regular clinical practice. The 24 clinical vignettes included 83.3% female patients, with a mean of 36.5±17.9 years of age. Active SLE organ involvement included: skin rashes (20.8%); arthritis (12.5%); nephritis (12.5%); thrombocytopenia (12.5%); cardiac/pulmonary involvement (12.5%); mucocutaneous vasculitis (8.3%); serositis (8.3%); neuropsychiatric lupus (8.3%). Systemic vasculitis, myositis, alopecia, hemolytic anemia, and leukopenia were each present in 4.2% of the vignettes. Hypocomplementemia and/or high anti-dsDNA were present in 75.0%. Twenty-one percent of the cases were in remission.All raters completed the survey, totaling 912 case assessments. Scores attributed by the raters ranged from 0.37 to 49.53 in SLE-DAS, 0 to 24 in SLEDAI-2K, and 0.0 to 3.0 in PGA. The interrater reliability was good for SLE-DAS and SLEDAI-2K, and moderate for PGA. The intra-rater reliability was excellent for SLE-DAS, and good for SLEDAI-2K and PGA (Table 1).Table 1:Interrater and intra-rater reliability of SLE-DAS, SLEDAI-2K and PGA.ICC (95%CI)Interrater reliabilityIntra-rater reliabilitySLE-DAS0.877 (0.807-0.934)0.908 (0.891-0.923)SLEDAI-2K0.812 (0.717-0.896)0.892 (0.871-0.909)PGA0.704 (0.578-0.828)0.900 (0.881-0.916)CI: confidence interval; ICC: intraclass correlation coefficient.ConclusionSLE-DAS presents high intra- and interrater reliability for measuring SLE disease activity. The high reliability of SLE-DAS is an important quality both in clinical practice and research, allowing consistent scoring among different clinicians including those who are not SLE experts.References[1] Jesus D, Matos A, Henriques C, et al. Derivation and validation of the SLE Disease Activity Score (SLE-DAS): a new SLE continuous measure with high sensitivity for changes in disease activity. Ann Rheum Dis 2019;78:365-71.[2] Piga M, Chessa E, Morand EF, et al. Physician Global Assessment International Standardisation Consensus in Systemic Lupus Erythematosus: the PISCOS study. Lancet Rheumatol 2022;4:e441-e449.AcknowledgementsBeatriz Mendes and Carolina Mazeda contributed equally and share first authorship.Disclosure of InterestsNone Declared.

Abstract Background Barraquer-Simons syndrome (BSS) is a rare, acquired form of lipodystrophy characterized by progressive loss of upper body subcutaneous fat, which affects face, upper limbs, and trunk. The pathogenesis of the disease is not entirely known and may involve autoimmune mechanisms. Aim This study aimed to provide a comprehensive picture of the clinical, immunological, and metabolic features of a large cohort of patients with BSS. Our primary objectives included the validation of existing diagnostic tools, the evaluation of novel diagnostic approaches, and the exploration of potential disease triggers or genetic predispositions. Subjects and Methods Twenty-six patients were diagnosed with BSS based on accepted criteria defined by international guidelines. Anthropometric parameters, biochemical tests, organ- and non-organ-specific autoantibodies, HLA status, and screening of the LMNB2 gene were performed. Results Patients were predominantly females (73%); fat loss occurred mostly during childhood (77%) at a median age of 8 years. Among various anthropometric measures, the ratio between the proportion of fat mass in upper limbs and lower limbs showed the best predictive value for diagnosis. A total of 11.5% of patients had diabetes, 34.6% dyslipidemia, and 26.9% hepatic steatosis. Seventy-five percent of children and 50% of adults had C3 hypocomplementemia; 76% of patients were positive for 1 or more autoantibodies. HLA-DRB1 11:03 had higher allelic frequencies compared with the general population. A single variant in the LMNB2 gene was found in 1 patient. Conclusion BSS has a childhood onset and is often associated with autoimmune diseases. Skinfold thickness measurements and fat assessment by dual energy X-ray absorptiometry are useful tools to identify the disease. C3 hypocomplementemia and the presence of autoantibodies may be used as additional diagnostic supportive criteria but the prevalence of C3 hypocomplementemia may be lower than previously reported.

Background:The ACR-1997, SLICC-2012 and EULAR/ACR-2019 classification criteria have high sensitivity and specificity for SLE, yet they classify non-overlapping groups of patients suggesting that they can be supplemented with additional features to improve their diagnostic performance.Objectives:To identify criteria and non-criteria manifestations that are significantly associated with SLE in clinical practice and can be used to complement the existing sets of classification criteria.Methods:Individual items from all three classification criteria (ACR-1997, SLICC-2012, EULAR/ACR-2019) and non-criteria features were analyzed in a randomly selected sample of 800 adults diagnosed with SLE or control rheumatologic diseases (1:1 ratio). The classification performance of each set of criteria was analyzed in combination with complementary features; multivariable least absolute shrinkage and selection operator (LASSO) logistic regression was performed for feature selection. We calculated the diagnostic odds ratio (DOR) of the criteria and the additional features retained in each model.Results:Τhe EULAR/ACR-2019 and SLICC-2012 criteria have increased accuracy for SLE classification as compared to the ACR-1997 criteria (univariate DOR: 243.2 and 157.3 versus 78.8, respectively). In multivariable regression based on the ACR-1997 criteria, inclusion of additional features such as maculopapular rash, alopecia and hypocomplementemia significantly enhanced the model predictive capacity (area under the curve [AUC]: 0.95 versus 0.87 of the ACR-1997 criteria alone). Similar analysis based on the SLICC-2012 and EULAR/ACR-2019 criteria identified photosensitivity as an additional criterion significantly associated with SLE (multivariable DOR: 5.4 and 9.4, respectively). Accordingly, models including photosensitivity had superior predictive capacity over the criteria-only models (AUC: 0.94 versus 0.91 for SLICC-2012, 0.96 versus 0.91 for EULAR/ACR-2019). Furthermore, non-criteria features including Raynaud’s/livedo reticularis, anti-RNP antibodies, splenomegaly and myocarditis were independently associated with SLE thus enhancing further the predictive capacity of criteria-based models.Conclusion:We identified a number of criteria and non-criteria features which can be used in combination with the existing sets of criteria to increase classification of SLE patients in clinical practice. Photosensitivity could be considered as an additional feature to improve sensitivity of the recent classification criteria.Disclosure of Interests:Christina Adamichou: None declared, Irini Genitsaridi: None declared, Dionysis Nikolopoulos: None declared, Alessandra Bortoluzzi: None declared, Antonis Fanouriakis Paid instructor for: Paid instructor for Enorasis, Amgen, Speakers bureau: Paid speaker for Roche, Genesis Pharma, Mylan, Eleni Kalogiannaki: None declared, Emmanouil Papastefanakis: None declared, Irini Gergianaki: None declared, Prodromos Sidiropoulos: None declared, Dimitrios Boumpas: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis

Background:Defining remission for SLE as a suitable target for a treat to target (T2T) approach has been a major challenge in the past years. A few years back, four definitions of remission were presented by the international DORIS task force.[1] Parameters included in the definition are clinical activity (cSLEDAI), steroid dose, immunosuppressive therapy, serology and physician global assessment (PGA). In particular the PGA, its threshold and general utility have been and still are discussed controversially.Objectives:It was our aim to evaluate the added value of PGA in remission assessment.Methods:In this monocentric cross-sectional study, patients with SLE according to the 1997 American College of Rheumatology (ACR) criteria were enrolled and assessed between September 2016 and December 2017. Two different definitions of remission were applied. The internationally accepted DORIS remission and a modified DORIS remission excluding PGA. Factors influencing PGA were assessed in the entire cohort. Regression analyses were used to assess differences between patients in DORIS and modified DORIS remission.Results:A total of 233 patients were included (87.6% female). 98 patients (41.9%) fulfilled any of the four DORIS remission definitions, while 154 patients (66.1%) were in any modified remission in which PGA was excluded. In general, PGA rating was associated with disease activity (clinical SLEDAI; p=<0.0001), depression (Center for Epidemiological Studies Depression Scale; p=0.049), pain reported by the patient (numeric rating scale; p=<0.0001) and hypocomplementemia (p=<0.0001). Damage (SLICC damage index, SDI) did not influence PGA (p=0.98). Both, DORIS and modified DORIS remission were associated with lower damage (p=0.026; p=0.003), lower pain on NRS (p=0.001; p=0.013), normal complement (p=0.0005; p=0.005) and better illness perception (p=0.006; p=0.023). Patients in modified DORIS remission had a tendency for more immunosuppressive therapy (p=0.046).Conclusion:Exclusion of PGA in remission assessment led to an increased number of patients in remission. Clinical parameters and factors associated with DORIS remission vs. modified DORIS remission were similar, hence the added value of PGA in our cohort regarding remission assessment is questionable. The use and especially the correct threshold of PGA for remission still has to be discussed.References:[1]van Vollenhoven, Ronald; Voskuyl, Alexandre; Bertsias, George; Aranow, Cynthia; Aringer, Martin; Arnaud, Laurent et al. (2017): A framework for remission in SLE: consensus findings from a large international task force on definitions of remission in SLE (DORIS). In: Annals of the rheumatic diseases 76 (3), S. 554–561. DOI: 10.1136/annrheumdis-2016-209519.Disclosure of Interests:Johanna Mucke: None declared, Christina Duesing: None declared, 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.

ObjectivesTo assess the risk of flare and damage accrual after tapering glucocorticoids (GCs) in modified serologically active clinically quiescent (mSACQ) patients with systemic lupus erythematosus (SLE).MethodsData from a 12-country longitudinal SLE cohort, collected prospectively between 2013 and 2020, were analysed. SLE patients with mSACQ defined as the state with serological activity (increased anti-dsDNA and/or hypocomplementemia) but without clinical activity, treated with ≤7.5 mg/day of prednisolone-equivalent GCs and not-considering duration, were studied. The risk of subsequent flare or damage accrual per 1 mg decrease of prednisolone was assessed using Cox proportional hazard models while adjusting for confounders. Observation periods were 2 years and censored if each event occurred.ResultsData from 1850 mSACQ patients were analysed: 742, 271 and 180 patients experienced overall flare, severe flare and damage accrual, respectively. Tapering GCs by 1 mg/day of prednisolone was not associated with increased risk of overall or severe flare: adjusted HRs 1.02 (95% CI, 0.99 to 1.05) and 0.98 (95% CI, 0.96 to 1.004), respectively. Antimalarial use was associated with decreased flare risk. Tapering GCs was associated with decreased risk of damage accrual (adjusted HR 0.96, 95% CI, 0.93 to 0.99) in the patients whose initial prednisolone dosages were >5 mg/day.ConclusionsIn mSACQ patients, tapering GCs was not associated with increased flare risk. Antimalarial use was associated with decreased flare risk. Tapering GCs protected mSACQ patients treated with >5 mg/day of prednisolone against damage accrual. These findings suggest that cautious GC tapering is feasible and can reduce GC use in mSACQ patients.

Background:Lupus nephritis (LN) is a severe manifestation of systemic lupus erythematosus (SLE) and can be present in both early-onset (age less than 50) (eSLE) and late-onset lupus (age greater than 50) (ltSLE) patients, with distinct differences in clinical features, prognosis, and treatment strategies. Understanding these differences is crucial for optimizing outcomes of LN in different age groups.Objectives:We performed a systematic review and meta-analysis to evaluate the differences in renal manifestations (lupus nephritis, end stage renal disease (ESRD), proteinuria, nephrotic syndrome) in ltSLE patients compared to eSLE patients.Methods:Literature search was performed using PubMed, Web of Science and the Cochrane Library. Cohorts, case-control, and population-based studies have been included. PRISMA 2020 guidelines and checklist were used (Flow chart attached). Studies were included if they investigated the frequency of LN in ltSLE patients and excluded if they did not include an eSLE control group. The diagnosis of SLE and LN (persistent proteinuria greater than 500 mg/day or cellular casts) was based on ACR 1997 revised classification criteria. Two authors independently reviewed all articles and evaluated the data for consistency between the abstracts, tables, and text. Forest plot was used to compare odds ratios (95% CI) of renal manifestations by age groups. Study heterogeneity was assessed using I2. All analyses were performed using R Statistical Software meta package (v4.2.1; R Core Team 2022)Results:45 eligible studies comprising 22,511 SLE patients (19307 eSLE and 3204 ltSLE) were included in the analysis. The frequency of LN was more common in eSLE group compared to ltSLE (OR: 2.37, 95% CI: 2.16-2.61, p<0.0001) (Figure 1). Further analysis of 12 studies showed that proteinuria was more frequent in eSLE when compared to ltSLE (OR: 1.9, 95%CI: 1.66-2.18, p=0.0001) while data from 9 studies showed that nephrotic syndrome was more frequent in eSLE than in ltSLE (OR: 4.42, 95%CI: 2.85-6.84), p<0.0001). Hypocomplementemia was more common (OR: 2.63, 95%CI: 2.29-3.02, p<0.0001) and positive dsDNA was more frequent in the eSLE group (OR: 1.33 95%:1.18-1.51, p<0.0001). The development of ESRD was reported in 12 studies. ESRD rate was higher in the eSLE than in ltSLE, but the difference was not statistically significant (OR: 1.45, 95% CI: 0.95-2.23, p=0.08).Conclusion:In this study, overall frequencies of LN, nephrotic syndrome, proteinuria, hypocomplementemia and elevated dsDNA were more common in eSLE patients compared to ltSLE. ESRD development tended to be higher in eSLE patients. These findings underscore the need for a high index of suspicion for LN in an older SLE patient population, particularly considering the high frequency of comorbid conditions such as diabetes, hypertension and vascular disease that can contribute to renal dysfunction.Figure 1.Forest plot for OR of LN in early and late onset SLE groups.Figure 1.Prisma Flow Chart.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:Sarah Abi Doumeth: None declared, Marina Magrey Yes, however, not related to SLE.Novartis, Eli, Lilly, Pfizer, Abbvie, Janssen, UCB pharma., Niraj Desai: None declared, Omer Pamuk: None declared.