tbuckley/cabot-search huggingface dataset:
AB0655 FAMILIAL AGGREGATION OF AUTOIMMUNE DISEASES IN PATIENTS WITH RHEUMATOID ARTHRITIS- A ROMANIAN STUDY
Authors: C. Alexandru, M. Bojinca, S. Bezedica, I. Ancuta, C. Dumitrașcu, C. Cobilinschi, C. C. Iorgus, A. Bobirca
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Rheumatoid Arthritis (RA) is a chronic, autoimmune rheumatic disease, frequently affecting women. Evidence suggests that a family history of autoimmune diseases (ADs) increases the risk of RA. Overall, family aggregation studies can bring valuable information regarding the genetic background of this autoimmune disease. <h3>Objectives:</h3> To determine the frequency and disease-related characteristics of familial autoimmune diseases co-aggregation in patients with rheumatoid arthritis. <h3>Methods:</h3> In this cross-sectional study, we registered 160 consecutive patients diagnosed with RA, according to the 2010 ACR/EULAR Classification Criteria, from a Rheumatology Department in Bucharest, between June 2022 and September 2023. The data regarding the presence of ADs among first to third degree relatives, as well as RA features were collected at hospital admissions, using surveys. For the comparative analysis, the cohort was divided into two subgroups: the patients with at least one relative with an AD and the patients without affected family (Group A and Group B, respectively). <h3>Results:</h3> The mean(SD) age of the cohort was 54.8 (14.7) years old, female sex being predominant (88.1%). The disease's duration had a mean of 14.0 (10.9) years. The frequency of familial co-aggregations was 33.8% as follows: 26.3% had first degree relatives with ADs, 10% had second degree relatives and 3.1% had third degree relatives affected. The most common AD diagnosed among relatives was rheumatoid arthritis, followed by autoimmune hypothyroidism. Moreover, the frequency of patients with relatives diagnosed with RA was 10.0% for first degree relatives, 5.6% for second degree relatives and 1.3% for third degree relatives. First degree relatives were most often identified, with a total of 10 pairs of parent-child and 6 pairs of sibling-sibling diagnosed with RA. The patients from Groups A had more frequent poliautoimmunity, RA seropositivity and were more frequently treated with biological therapy than Group B (Table 1). Moreover, patients with at least one relative with AD were significantly younger at disease onset, mean (SD) of 35.8 (15.1) years old, compared with 43.3 (15.9) years old (p=0.007). <h3>Conclusion:</h3> One third of RA patients appear to have a familial aggregation of ADs, with first degree relatives, particularly parent-child relationships, being more significant. Furthermore, patients with familial aggregation are predisposed to develop the disease at a younger age. <h3>REFERENCES:</h3> [1] Kuo CF et al; Familial aggregation of rheumatoid arthritis and co-aggregation of autoimmune diseases in affected families: a nationwide population-based study. Rheumatology (Oxford). 2017 Jun 1;56(6):928-933. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
SGLT2 Inhibitor Therapy in Patients With Transthyretin Amyloid Cardiomyopathy
Authors: Aldostefano Porcari, Francesco Cappelli, Christian Nitsche, Daniela Tomasoni, Giulio Sinigiani, Simone Longhi, Luca Bordignon, Ahmad Masri, Matteo Serenelli, Marcus Urey, Beatrice Musumeci, Alberto Cipriani, Marco Canepa, Roza Badr-Eslam, Christina Kronberger, Cristina Chimenti, Mattia Zampieri, Valentina Allegro, Yousuf Razvi, Rishi Patel, Adam Ioannou, Muhammad U. Rauf, Aviva Petrie, Carol Whelan, Michele Emdin, Marco Metra, Marco Merlo, Gianfranco Sinagra, Philip N. Hawkins, Scott D. Solomon, Julian D. Gillmore, Marianna Fontana
Journal: Journal of the American College of Cardiology (2024)
POS0893 PANEL OF SERUM BIOMARKERS FOR DIFFERENTIAL DIAGNOSIS OF IDIOPATHIC INTERSTITIAL LUNG DISEASE AND INTERSTITIAL LUNG DISEASE-SECONDARY TO AUTOIMMUNE RHEUMATIC DISEASE
Authors: E. Conticini, P. Cameli, C. V. Cotton, J. Lamb, L. Bergantini, S. Gangi, G. Montuori, S. Sugden, L. Spencer, B. Frediani, R. P. New, H. Chinoy, E. Bargagli, M. D’alessandro
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Interstitial lung disease (ILD) may complicate the course of autoimmune rheumatic disease (ARD) and diagnostic biomarkers are needed. Krebs von den Lungen-6 (KL-6), ferritin (FER) and interleukin 6 (IL-6) have been involved in the ILD development. <h3>Objectives:</h3> Our study aimed to compare KL-6, FER, IL-6 and soluble mesothelin-related peptide (SMRP) concentrations in a cohort of primary and secondary-ILD. <h3>Methods:</h3> 3169 patients were enrolled in the "UK Biomarkers in Interstitial Lung Disease (UK-BILD) Study". We selected patients affected by ARD-ILD and idiopathic ILD (usual interstitial pneumonia-idiopathic pulmonary fibrosis and fibrotic non-specific interstitial pneumonia). Serum marker concentrations were measured through chemiluminescent assays (Fujirebio Europe, Ghent, Belgium). <h3>Results:</h3> 1013 patients were selected for the study: 520 (51.3%) had idiopathic ILD and 493 (48.7%) ARD-ILD. Idiopathic ILD patients displayed higher KL-6 values than ARD-ILD (p=0.0002). FER and SMRP, though within normal ranges, were significantly higher in idiopathic ILD (p&lt;0.0001). Logistic regression showed good sensitivity (69.4%) and specificity (80.4%) selecting the variables FER and KL-6 concentrations, age and gender-male correlated with a diagnosis of idiopathic ILD. These findings enabled us to build a model with an accuracy of 0.755 for differential diagnosis of idiopathic ILD and ARD-ILD based on the following variables: age, gender-male, FER, SMRP, smoking history, Asian and Afro-Caribbean ethnicity, IL-6 and KL-6. Logistic regression showed that the variables that best correlated with a diagnosis of idiopathic ILD were FER and KL-6 concentrations, age and gender-male. <h3>Conclusion:</h3> Our multicentre, retrospective study is the first and largest to evaluate an extended panel of serum biomarkers in patients with different types of ILD, including ARD-ILD. We observed normal serum concentrations of IL-6, FER and SMRP in both groups, whereas KL-6 appeared above normal cut-off in most patients but was significantly higher in the idiopathic ILD group. Our study showed the excellent diagnostic value of KL-6 for detecting ILD, which irrespective of the final diagnosis and extent of disease, is always elevated and is a reliable biomarker of lung fibrosis in various diseases, ranging from idiopathic to autoimmune forms. Ours is the first study to report high serum SMRP concentrations in idiopathic ILD. In this context, combination of serum markers and clinical data, as seen in our cohort, may lead to a further improvement in diagnostic accuracy for ILD. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1086 A PROMISING GLYCOBIOMARKER TO PREDICT CLINICAL CHARACTERISTICS AND DISEASE ACTIVITY OF PATIENTS WITH JUVENILE LUPUS ERYTHEMATOSUS PATIENTS: PRELIMINARY RESULTS
Authors: D. Santos Oliveira, I. Alves, S. Ganhão, F. R. Martins, R. Nicolau, T. Beirão, F. Aguiar, M. Rodrigues, S. Martins, R. Farinha, S. Pinho, I. Brito
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Modifications in protein glycosylation have been shown to significantly contribute to the immunopathogenesis of various immune-mediated diseases. However, a clinical glycobiomarker to predict the development and the severity of juvenile lupus erythematosus (jLES) is still unknown. <h3>Objectives:</h3> To explore the role of a promising clinically-relevant glycobiomarker to predict clinical characteristics and disease activity in patients with jLES. <h3>Methods:</h3> Patients diagnosed with SLE before the age 21 years-old were included. Sociodemographic, clinical and laboratory data were collected namely age at diagnosis, response to pharmacological treatment and disease activity measured with Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Urine and blood samples from each patient were collected and, in the latter, serum was isolated. Protein and Neutrophil gelatinase-associated lipocalin (NGAL) levels were evaluated in urine samples and the glycobiomarker in blood samples. ELISA was performed to quantify the levels of the glycobiomarker. The cut-off that better classify the cohort was determined by Receiver Operating Curve (ROC) towards each clinical variable. Univariate binary logistic regression was used to explore possible associations between high levels of glycobiomarker and diagnosis/prognosis clinical variables. <h3>Results:</h3> A total of 36 patients with jLES, with a mean age of 24.2±7.7 years old and age at diagnosis of 13.9±3.8 years were included. Most patients were female (97.2%). Our results demonstrated that in patients with jLES, higher levels of glycobiomarker are associated with a later age at diagnosis, above 15 years old (OR 5.28, p=0.028) (Table 1) with 82.4% of sensitivity and 56.2% of specificity (Figure 1A). Moreover, higher levels of glycobiomarker also positively associate with higher levels of NGAL at diagnosis (OR 10.27, p=0.005) (Table 1) with 74% sensitivity and 50% specificity (Figure 1B). Other explored variables did not associate with glycobiomarker levels (Table 1). <h3>Conclusion:</h3> These findings showed that in patients with jSLE the glycobiomarker is positively associated with age of diagnosis and NGAL levels. Moreover, further longitudinal research with larger samples is needed to explore the association of this potential glycobiomarker with other clinical variables in order to be included in the diagnostic, monitoring and prognostic algorithm of jSLE. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1091 MORTALITY AND RISK OF END-STAGE RENAL DISEASE IN SYSTEMIC LUPUS ERYTHEMATOSUS: A RETROSPECTIVE COHORT STUDY ASSESSING TRADITIONAL CHINESE MEDICINE USERS
Authors: C. Y. Wei, H. H. Yu, K. H. Yu
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Systemic lupus erythematosus (SLE) frequently results in renal complications, significantly impacting both morbidity and mortality rates. Conventional drugs, including corticosteroids and disease-modifying antirheumatic drugs, while controlling disease activity, may lead to side effects and elevate the likelihood of severe infections. Consequently, many SLE patients also seek complementary therapies simultaneously, such as Traditional Chinese Medicine (TCM) [1]. <h3>Objectives:</h3> This retrospective cohort study aims to evaluate end-stage renal disease (ESRD) and mortality rates among patients with SLE utilizing Traditional Chinese Medicine. <h3>Methods:</h3> We utilized multi-institutional cohort data from the Chang Gung Research Database (CGRD) between January 1, 2005, and December 31, 2020. Patients were followed until the end of 2022, occurrence of ESRD, or death. TCM users were identified as SLE patients who visited Chinese medicine clinics at least twice and received oral TCM prescriptions. Two-stage propensity score matching (PSM) was applied to create a baseline characteristics-matched (age, sex, comorbidities, conventional drugs, creatinine, C3, C4, anti-dsDNA) cohort, selecting a ratio of 1:2 for the TCM and non-TCM groups. Cox proportional hazard regression and Kaplan-Meier methods were used to assess mortality and ESRD rates. <h3>Results:</h3> PSM successfully balanced the differences between the TCM and non-TCM groups, resulting in a cohort of 5637 patients (1879 TCM users and 3758 non-TCM users). The mortality rate was 1.31 per 100 person-years for the TCM group and 2.52 for the non-TCM group. After adjusting for confounding covariates, the hazard ratio of mortality for the TCM group was 0.72 (95% CI 0.62-0.84, p&lt;0.0001). The TCM group exhibited significantly higher survival rates (log-rank test p&lt;0.0001; Figure 1). While ESRD rates showed no significant difference, a more stringent inclusion criterion (TCM usage ≥30 days) revealed a consistent, albeit not statistically significant, trend of reduced ESRD risk throughout the 0.5 to 4-year follow-up periods (Table 1). <h3>Conclusion:</h3> Patients with SLE using TCM demonstrated lower mortality rates, emphasizing a potential benefit of TCM in managing SLE. Additionally, a trend towards reduced ESRD risk emerged with prolonged TCM use, suggesting a possible protective effect over an extended follow-up period up to the fourth year. <h3>REFERENCES:</h3> [1] Chou CT. Alternative therapies: what role do they have in the management of lupus? Lupus. 2010 Oct;19(12):1425-9. <h3>Acknowledgements:</h3> The authors thank Po-Chuan Ko for the statistical assistance and wish to acknowledge for statistical and data analysis asistance and interpretation support by the Center for Big Data Analytics and Statistics, Chang Gung Memorial Hospital, Linkou. <h3>Disclosure of Interests:</h3> <b>None declared.</b>
OP0249 A GLIMPSE OF MRI DEEP WHITE MATER HYPERINTENSITIES IN SYSTEMIC SCLEROSIS PATIENTS
Authors: S. Xu, X. Tong, H. He, J. Zhou, Q. Wang, D. Xu, X. Zhao, M. LI, X. Zeng
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> As a systemic autoimmune disease, Systemic Sclerosis (SSc) can involve multiple organs including thebrain. White matter hyperintensities (WMHs), including periventricular hyperintensity (PVH) and Deep white-matter hyperintensity (DWMH), are abnormal findings on brain magnetic resonance imaging (MRI). RecentlyDWMHs are considered to be affected by ischemia-hypoperfusion, which may reflect vascular changes. <h3>Objectives:</h3> This study attempts to evaluate the volumes of DWMHs in SSc patients and health controls. <h3>Methods:</h3> Forty-nine patients without central nervous system symptoms or histories and twenty-four health controlswith matched age and sex were included in this study. Brain MR imaging was performed on a 3T scanner(Ingenia CX, Philips Healthcare, The Netherlands). T1-weighted imaging was obtained to calculate the whole white matter (WWM) volumes by using MRICloud automated segmentation pipelines. WMHs were evaluatedon fluid-attenuated inversion recovery (FLAIR) images using Fazekas scores (Periventricular hyperintensity 0-3, Deep white matter hyperintensity 0-3). The volumes of DWMHs and WMHs were calculated by manuallydrawing regions of interest (Figure 3) in 3D Slicer open-source software (version 5.4.0, available atwww.slicer.org). Volume ratios (including VR1= volume DWMHs/ volume WWM, VR2= volume WMHs/ volumeWWM, VR3= volume DWMHs/ volume WMHs) of each individual were compared between groups. Thestatistical analyses were conducted using SPSS 27.0 (SPSS Inc. Chicago, IL, USA). Wilcoxon Mann-Whitneytest was applied to compare the differences between groups and Spearman correlation coefficients (rs) formeasuring the associations among quantitative variables. <h3>Results:</h3> VR1 and VR3 were significantly larger in SSc patients than in health controls (P&lt;0.05) (Figure 1 and Figure 2).Significant positive correlations were found between total Fazekas scores (PVH+DWMH) and VR1(rs =0.83,P&lt;0.01), age and VR1 (rs =0.599, P&lt; 0.01) (Table 3). The FLAIR-MRI showed DWMHs in 38 patients (77.5%) and17 health controls (70.83%). While Fazekas scores and VR2 showed no significant differences between SScpatients and health controls (Table 1 and Table 2). <h3>Conclusion:</h3> Compared to healthy people, the VR1 and VR3 in Systemic Sclerosis patients without central systemicsymptoms or histories are larger. This suggested that in the central nervous system, vascular lesions mayappear in the early stage before symptoms appear, and SSc patients are more likely to have WMH in thedeep white matter rather than around the periventricular. VR1 may be a potential sensitive marker orcriterion of the early SSc central nerve system involvement evaluation. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1106 EFFICACY AND SAFETY OF BELIMUMAB IN SYSTEMIC LUPUS ERYTHEMATOSUS: MONOCENTRE STUDY
Authors: F. Riccio, M. Annunziata, M. Brancaccio, M. L. Marchesano, A. Milone, C. Parlati, M. Patrone, D. Mauro, S. Fasano, F. Ciccia, G. Cuomo
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Over the past few years, the efficacy of several biological drugs for the treatment of Systemic Lupus Erythematosus has been evaluated. The efficacy of belimumab in reducing disease flares and organ damage is now strongly established [1]. <h3>Objectives:</h3> We will analyse the efficacy of belimumab in reducing disease activity after 3 and 6 months of treatment. <h3>Methods:</h3> All the 74 patients from the Lupus Clinic of our Rheumatology Unit who received belimumab between 2014 and 2023 were selected for this study. 69% of the patients were also taking another DMARD (azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate), while 75% were also taking hydroxychloroquine. Clinical and epidemiological date were assessed at baseline and 3 and 6 months after first belimumab administration, with SLEDAI-2K, C3 and C4, anti-dsDNA antibodies, proteinuria over 24 hours and steroid dosage being particularly highlighted. Adverse events were also analysed. <h3>Results:</h3> 94% of the patients selected were female, mean age 41±13.1 years, median 39 (range 15-83). The mean disease duration was 15.2±9 years, median 14 (range 3-37). The mean prednisone dosage was 9.3±5.7 mg, median 10 mg (range 0-25) and the mean SLEDAI-2K score was 7.3±3.7, median 8 (range 0-16). 32.8% of the patients had biopsy-confirmed Lupus nephritis (7% class II, 49% class III, 43% class IV, 1% class V). 27.74% of patients had arthritis, 39% muco-cutaneous manifestations, 8% serositis, 32.8% haematological changes and 2.73% neuropsychiatric involvement. 7.6% of the patients had C3 and C4 reduced. The mean anti-dsDNA values were 113.5±153 IU/mL, median 79 (range 0-1000). Clinical features at T0, T3 and T6 are summarised in Table 1. The data have confirmed a significant reduction of SLEDAI-2K, C3 and C4, anti-dsDNA antibodies, proteinuria and prednisone dosage from the first months of treatment. No significant differences were found regarding ESR, CRP, haemoglobin and creatinine. Adverse events are summarised in the Table 2. <b>Table 1</b> <b>Table 2</b> <h3>Conclusion:</h3> Belimumab is effective in reducing disease activity already after 3 months of treatment, acting on the different clinical features of the disease, especially proteinuria. Adverse events are extremely rare. Therefore, the use of this drug is crucial in ensuring rapid steroid-sparing. <h3>REFERENCES:</h3> [1] Cureus.2022 Jun 13;14(6):e25887 <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1085 CONCENTRATIONS OF SUBCUTANEOUSLY ADMINISTERED BELIMUMAB IN HUMAN BREAST MILK IN A WOMAN WITH SYSTEMIC LUPUS ERYTHEMATOSUS, A CASE REPORT
Authors: B. Blomjous, M. De Boer, M. Van Weissenbruch, K. Laan, T. Rispens, A. E. Voskuyl, I. Bultink
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Belimumab is a human IgG1-lambda monoclonal antibody that inhibits the B-cell survival factor BLyS. It is used in patients with active systemic lupus erythematosus (SLE). <h3>Objectives:</h3> Since data on the safety of belimumab during breastfeeding is scarce, we investigated belimumab concentrations measured in breast milk of a woman with SLE initiating treatment with subcutaneous belimumab due to a lupus flare three months after delivery. <h3>Methods:</h3> Our patient stopped giving breastfeeding when she started on subcutaneously administered belimumab. During the phasing out of breastfeeding she pumped breast milk to prevent engorgement. Milk samples were collected two and four weeks after the initiation of belimumab. Belimumab concentrations were measured by using an in-house developed enzyme-linked immunoabsorbent assay (ELISA). <h3>Results:</h3> Belimumab concentrations measured in two breastmilk samples were 0.264 ug/mL at day 14 and 0.885 ug/mL at day 28 (Table 1). <h3>Conclusion:</h3> This case report shows that subcutaneously administered belimumab was transferred to breastmilk. The higher concentration at day 28 is probably caused by the low volume and long stasis of the breastmilk during phasing out. The concentrations measured in breast milk were much lower than those measured in serum of adult patients (around 70 ug/mL) [1]. The actual serum level of belimumab in the infant is supposed to be low, because of proteolysis in the infant's stomach and low intestinal absorption. However data on infant serum levels are not available and urgently needed in order to be able to weight the possible risk of transfer of belimumab to the infant against the positive effects of breastfeeding for mother and infant. <h3>REFERENCES:</h3> [1] Struemper, H., M. Thapar, and D. Roth, <i>Population Pharmacokinetic and Pharmacodynamic Analysis of Belimumab Administered Subcutaneously in Healthy Volunteers and Patients with Systemic Lupus Erythematosus.</i> Clin Pharmacokinet, 2018. <b>57</b>(6): p. 717-728. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> Birgit Blomjous: None declared, Marjon de Boer: None declared, Mirjam van Weissenbruch: None declared, Koen Laan: None declared, Theo Rispens: None declared, Alexandre E. Voskuyl: None declared, Irene Bultink Speaker fees: AstraZeneca, GSK, UCB, Consultancy fees: AstraZeneca, GSK, UCB, Sanofi Genzyme, Eli Lilly.