tbuckley/cabot-search huggingface dataset:
AB1230 SYSTEMIC SCLEROSIS: CLINICAL FEATURES AND HEALTH RELATED QUALITY OF LIFE AT GROOTE SCHUUR HOSPITAL, CAPE TOWN
Authors: B. Hodkinson, R. Daimari
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> There is no published research from sub-Saharan African on pain, fatigue or disability amongst persons living with systemic sclerosis (SSc). <h3>Objectives:</h3> To describe the clinical characteristics and health-related quality of life (HRQoL) of persons with SSc attending a state-sector academic hospital in South Africa (SA). <h3>Methods:</h3> A cross-sectional study of consenting outpatients (≥18 years; meeting ACR/EULAR 2015 SSc classification criteria). Clinico-demographic details and patient-reported outcome measures including the Brief Pain Questionnaire (BPI), Health Assessment Questionnaire (HAQ-DI), Functional Assessment of Chronic illness Therapy-Fatigue (FACIT-F) and the Hospital Anxiety (HADS-A) and Depression Scale(HADS-D) were collated. Patients with no or mild functional disability (HAQ-DI ≤0.5) were compared to those with moderate to severe disability (HAQ-DI&gt;0.5). <h3>Results:</h3> Of 77 participants, (67 females), the mean (SD) age at diagnosis was 44.1(12.4) and disease duration was 10.9(9.2) years. The majority (58%) were unemployed and 26% depended on a state disability grant. Half had diffuse disease (50.5%), the majority had Raynaud's (93.5%) and digital ulcers (64.9%), and the mean (SD) modified Rodnan skin score was 11.3(12.1). Gastroesophageal reflux was noted in 83.1% and 49.3% had interstitial lung disease. Positive ANA, anti- topoisomerase and anti-centromere antibodies were present in 75.3%, 19.4% and 13.0% respectively. The mean (SD) HAQ-DI was 1.2(0.8), indicating moderate to severe disability. The worst scores were seen in the "reach" domain, with 45% scoring &gt;2 ("with much difficulty") for reaching an object above the head. Moderate or severe pain was experienced by 44.2% and 18.2%, and moderate or severe fatigue in 52.7% and 12.7%. Severe anxiety or depression was noted in 28.6% and 24.6% respectively. Patients with functional disability had significantly worse fatigue, pain (both severity and interference scores), anxiety and depression scores, and were more likely to haver digital ulcers, musculoskeletal, lung or gastrointestinal involvement, although these clinical features did not reach statistical significance. <h3>Conclusion:</h3> Amongst indigent state-sector SScl patients, functional disability is prevalent and is significantly associated with fatigue, pain and poor mental health. Future studies will assess the effectiveness of interventions including occupational therapy and psychosocial therapy. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> Department of Medicine, Rheumatology University of Cape Town. <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1080 PREVALENCE AND OUTCOME OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH) AND MACROPHAGE ACTIVATION SYNDROME (MAS) IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS (SLE): A SYSTEMATIC REVIEW AND META-ANALYSIS
Authors: S. Piplani, J. Reet, S. T. Kolli, M. U. Tufail, V. Jelic, K. Chang, A. Srivatsava, M. Sakellakis, A. Aljareh, H. Kim, S. P. Kasire, S. Chaturvedi, P. K. Komminni, P. Kumari, M. Vukelic, V. R. Bejugam, M. Radulovic
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) are characterized by tissue inflammation due to immune system dysfunction. MAS is associated with autoimmune diseases and involves overstimulated immune cells, leading to excessive cytokine release. Despite its rarity, MAS is often under recognized, causing delayed treatment and increased morbidity. HLH, a potentially life-threatening condition, results from immune system overreaction, causing organ damage. There is few data interpreting prevalence and outcomes of MAS and HLH. <h3>Objectives:</h3> We aim to assess the prevalence and outcome of Hemophagocytic lymphohistiocytosis (HLH) and Macrophage activation syndrome (MAS) in Systemic Lupus Erythematosus (SLE) patients. <h3>Methods:</h3> A systemic search was conducted from electronic databases (PubMed/Medline, Cochrane Library, and Google Scholar) from inception to 5th November 2023. All statistical analyses were conducted in Review Manager 5.4.1. Studies meeting inclusion criteria were selected. A random-effect model was used when heterogeneity was seen to pool the studies, and the result was reported in prevalence and their corresponding 95% confidence interval (CI). Outcomes were assessed using qualitative analysis. <h3>Results:</h3> A total of six studies were included in our manuscript. Five studies were used to conduct quantitative anlaysis which showed prevalence of HLH (prevalence = 5% (0%, 11%); p = 0.04; I2 = 99%) and MAS (prevalence = 13% (2%, 23%); p = 0.02; I2 = 94%). Outcomes related to mortality, length of stay, treatment and other outcomes were analyzed using qualitative analysis. <h3>Conclusion:</h3> Our study showed a 5% prevalence of HLH and 13% prevalence of MAS in SLE patients which might be fatal and may result in increasing length of stay for the these patients. <h3>REFERENCES:</h3> [1] Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson LA, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW; HLH/MAS task force. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Ann Rheum Dis. 2023 Oct;82(10):1271-1285. doi: 10.1136/ard-2023-224123. Epub 2023 Jul 24. PMID: 37487610. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0086 COMBINATION THERAPY OF HIGH-DOSE INTRAVENOUS ANAKINRA AND BARICITINIB IN PATIENTS WITH CRITICAL COVID-19: PROMISING RESULTS FROM RETROSPECTIVE OBSERVATIONAL STUDY
Authors: M. Bektaş
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Several targeted therapies such as anakinra, tocilizumab, and janus kinase (JAK) inhibitors were found to be effective in patients with COVID-19 associated cytokine storm. <h3>Objectives:</h3> Herein, we aimed to evaluate the safety and efficacy of combination of high-dose intravenous anakinra treatment and baricitinib in patients with critical COVID-19. <h3>Methods:</h3> Study population consisted of patients with positive polymerase chain reaction and computer tomography findings compatible with COVID-19 as well as critical non-intubated illness requiring high flow nasal oxygen supplement. Hyperinflammatory state of the patients were scored according to COVID-19 hyperinflammatory syndrome score (cHIS score). All patients received background glucocorticoid treatment with methylprednisolone 80 mg/day intravenously. Anakinra was initiated in patients who did not respond to two days of corticosteroid treatment or concomitantly in high risky patients with 400 mg/day intravenously in two divided dose and gradually increased to 1600 mg/day (10 mg/kg/day) if necessary. In combination group, baricitinib was started in 2 mg/day and gradually increased to 8 mg/day in addition to steroid and anakinra in patients with unresponsive to anakinra at least three days. Age, gender, disease severity, and cHIS scores matched patients receiving anakinra defined as control group. <h3>Results:</h3> Data of 15 patients in combination group and 43 patients in control group were evaluated and included into the study. Of these patients 73.3% was male in combination arm and 72.1% in control group (p=0.9). Overall mortality was 46.7% (n=7) in combination arm and 69.8% (n=30) in control group although it was not statistically significant (p=0.1). Similarly, need of intubation was also lower in combination arm (46.7%) compared to control group (69.8%), it was not significantly different (p=0.1). ICU admission was significantly lower in combination (46.7%, n=7) arm than control group (76.7%, n=33) (p=0.03, Odds ratio [OR]:4.7). Development of severe infection (20%, n=3 vs 25%, n=9/36), pulmonary embolism (6.7%, n=1 vs 0), myocardial infarction (6.7%, n=1 vs 2.6%, n=1/38) and pneumothorax (13.3%, n=2 vs 2.6%, n=1/38) were not different between two groups (p=0.7, p=0.3, p=0.5 and p=0.2). In survival analysis, mortality rate was significantly lower in combination arm than control group (Log-Rank:p=0.04; Figure 1). <h3>Conclusion:</h3> Our study emphasizes no additional safety signal including severe infection, thrombosis, and cardiovascular events with combination group. Furthermore, combination therapy tended to reduce mortality in patients with critical COVID-19 in our study. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1039 CHARACTERIZATION OF A MULTICENTER COHORT OF PATIENTS WITH LUPUS NEPHRITIS UNDERGOING KIDNEY TRANSPLANTATION
Authors: Á. Egea-Fuentes, V. Calvo-Río, L. Martin-Sierra, S. Sandoval-Moreno, C. A. Egües-Dubuc, N. Viveros-Pérez, M. Caeiro-Aguado, A. Mayo-Juanatey, M. Retuerto-Guerrero, M. C. Osorio-Sanjuan, L. Sala-Icardo, R. Blanco
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Lupus nephritis (LN) is a potentially severe complication of systemic lupus erythematosus. (SLE). As described in the literature, between 10 and 20% of patients with LN develop end-stage renal disease (ESRD), in some cases requiring kidney transplantation (KT) [1]. <h3>Objectives:</h3> To characterize a cohort of patients with LN from 10 Spanish hospitals who underwent KT to determine the frequency of renal graft loss and the role that LN flare plays on the transplanted organ. <h3>Methods:</h3> We carried out a multicenter descriptive study of all patients undergoing KT for LN in 10 Spanish hospitals between 1980 and 2023. The information was obtained by reviewing the clinical history in physical and electronic format of each patient. Epidemiological and clinical data were collected upon diagnosis of SLE and LN and cases of renal graft loss and its causes were recorded. <h3>Results:</h3> We included 76 patients, mostly women (n= 59, 77.63%) with high blood pressure as the most frequent cardiovascular risk factor (n= 52, 68.42%) and predominant histological diagnosis of LN type IV (n= 32, 42.11%). The mean time elapsed between the start of renal replacement therapy (RRT) and completion of kidney transplant (KT) was 2.63 ± 3.07 years (<b>Table 1</b>). As shown in <b>Table 2</b>, both brain death and asystole were the most frequently recorded donation subtypes (n=28, 36.84% and n=27, 35.53% respectively). Kidney graft loss was observed in 27 patients (35.53%), the most frequent causes were acute rejection (n=6, 22.22%), chronic rejection (n=6, 22.22%), acute and chronic rejection (n=5, 18.52%) and graft thrombosis (n=4, 14.81%). The outbreak of LN on the graft was the cause of organ loss in 3 patients (11.11%). <h3>Conclusion:</h3> Despite concerns about LN recurrence after KT, our data suggest that graft recurrence of LN is not a common cause of organ loss. <h3>REFERENCES:</h3> [1] ZHANG L, LEE G, LIU X et al.: Long-term outcomes of end-stage kidney disease for patients with lupus nephritis. Kidney Int 2016; 89: 1337-45. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> Á. Egea-Fuentes Abbvie, Pfizer, Janssen, Lilly, Novartis, UCB, Gebro, GSK, Amgen and Galápagos, V. Calvo-Río Abbvie, Lilly, Grünenthal, AMGEN, MSD, Novartis, Galápagos, Vifor, GSK, Otsuka and Janssen, L. Martin-Sierra: None declared, S. Sandoval-Moreno: None declared, C.A. Egües-Dubuc: None declared, N. Viveros-Pérez: None declared, M. Caeiro-Aguado: None declared, A. Mayo-Juanatey: None declared, M. Retuerto-Guerrero: None declared, M.C. Osorio-Sanjuan: None declared, L. Sala-Icardo: None declared, Ricardo Blanco: None declared.
AB1025 IMPACT OF LUPUS NEPHRITIS ON NEUROPSYCHIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS: CLINICAL INSIGHTS
Authors: P. J. Fang, P. H. Kuo, S. Y. Lyu, H. C. Chen, F. C. Liu, D. M. Chang, C. C. Lu
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Systemic lupus erythematosus (SLE) is an autoimmune disorder with diverse organ involvement, notably affecting the kidneys and nervous system. Lupus nephritis (LN) and neuropsychiatric SLE (NPSLE) are severe complications associated with increased morbidity and mortality. This retrospective study analyzes data from SLE patients with newly diagnosed NPSLE to explore the correlation between LN and NPSLE, focusing on clinical outcomes and potential predictive factors. <h3>Objectives:</h3> This article aims to clarify the correlation between LN and NPSLE, specifically focusing on how kidney inflammation influences distal organs, particularly the brain, in individuals with SLE. <h3>Methods:</h3> We retrospectively reviewed medical records from SLE patients with NPSLE at Tri-Service Hospital from November 2005 to December 2023. Data on demographic information, clinical manifestations, laboratory tests, Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI), complications, treatment, and mortality were collected. <h3>Results:</h3> Our study included 48 NPSLE patients, with 32 meeting the criteria for LN diagnosis. NPSLE patients were categorized into LN+ (with lupus nephritis) and LN− (without lupus nephritis) groups. The LN+ group had a higher SELENA-SLEDAI score (p=0.001). Regarding hospitalization, the LN+ group (mean: 26.3 days) exceeded the LN− group (mean: 11.9 days) (p=0.001). The LN+ group also had a higher percentage of intensive care unit (ICU) admission (40.6% vs. 6.3%, p=0.018) and mechanical ventilation use (31.3% vs. 0.0%, p=0.020). Although the LN+ group showed higher infectious complications (46.9%) compared to the LN− group (18.8%), it did not reach statistical significance (p = 0.058). However, the LN+ group's elevated mortality rate (25.8%) achieved statistical significance compared to the LN− group (0.0%) (p = 0.038). <h3>Conclusion:</h3> This study underscores the substantial impact of LN on the clinical outcomes of NPSLE, revealing increased lupus activity, prolonged hospitalization, higher ICU admissions, greater use of mechanical ventilation, and a higher mortality rate in the LN+ group compared to the LN− group. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> Statistics consultant: En Chao. <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1060 ASSOCIATION BETWEEN TRIGLYCERIDE-GLUCOSE INDEX AND CAROTID ATHEROSCLEROSIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A CROSS-SECTIONAL STUDY
Authors: Z. Zhang, W. Xie
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Patients with systemic lupus erythematosus (SLE) are at substantial increased risks of cardiovascular morbidities that have become leading cause of death. The triglyceride-glucose (TyG) index, proposed as a surrogate marker for insulin resistance, appeared to be a good indicator of clinically overt cardiovascular events and subclinical atherosclerosis in general population. However, there is no evidence available on the correlation of TyG index with the occurrence of clinical and subclinical cardiovascular diseases in SLE population. <h3>Objectives:</h3> We aimed to investigate the association between TyG index and carotid atherosclerosis in SLE patients. <h3>Methods:</h3> This cross-sectional study in a tertiary care hospitals included 330 eligible consecutive SLE patients receiving carotid ultrasonography with integrated TyG index, calculated as ln [fasting triglycerides (mg/dL)×fasting glucose (mg/dL)/2]. According to the TyG index tertiles, the patients were categorized into three groups. Logistic regression models were applied to analyze the association of TyG index as continuous variables and tertiles with carotid atherosclerosis and carotid artery plaque. <h3>Results:</h3> Of 330 SLE patients included, 97.1% were female, with a mean age of 39.1±12.7 years and median disease duration of 95 (38-170) months. The frequency of carotid atherosclerosis was 10.3% (34/330) and the mean TyG index was 8.49±0.54. As compared with those without carotid atherosclerosis, patients with carotid atherosclerosis had significantly higher TyG index (8.87±0.82 vs. 8.45±0.48, p&lt;0.001) (Figure 1). With increases in TyG index tertiles, the frequency of carotid atherosclerosis was increased, showing 3.7% for tertile 1, 9.1% for tertile 2 and 17.7% for tertile 3 (P=0.003). Multivariate logistic analyses showed that each 1-unit increase in TyG index was significantly associated with prevalent carotid atherosclerosis (unadjusted OR 3.84 (1.79-8.26); fully-adjusted OR 4.40 (1.52-12.69)). The unadjusted and fully-adjusted OR for occurrence of carotid atherosclerosis were 5.54 (1.83-16.80) and 4.19 (1.16-15.23) in patients with tertile 3 compared to patients with tertile 1 of TyG index (P for trend 0.006 and 0.042, respectively). Similar findings were found for the outcome of carotid artery plaque. <h3>Conclusion:</h3> The present study suggested that an elevated TyG index was associated with a higher risk of carotid atherosclerosis, independent of traditional cardiovascular risk factors and SLE-related factors. With the advantages of being simple, accessible and reliable, TyG index can be a useful tool for cardiovascular risk assessment for SLE patients in daily practice. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB1084 THE PROGNOSIS OF MIXED-TYPE LUPUS NEPHRITIS DEPENDS ON MORPHOLOGICAL CHANGES DUE TO EPITHELIAL DEPOSITS
Authors: R. Sakai, T. Kosaka, T. Aoki, S. Yoshinaga, A. Shibata, T. Kurasawa, T. Kondo, K. Amano
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> The combined proliferative and membranous lupus nephritis (mixed-type LN) is defined as class III/IV lesion and diffusely distributed membranous lesion (class V) using light microscopy (LM) or immunofluorescence (IF) in ISN/RPS 2003, and was reported with a poor prognosis. <h3>Objectives:</h3> In the renal outcome of mixed-type LN on complete renal remission (CRR) and end-stage renal disease (ESRD) three years after biopsy, we examined the differences between LM and IF, which were the rationale for concomitant membranous lesions. <h3>Methods:</h3> During 2005-2022, we retrospectively evaluated 43 cases of mixed-type LN divided into membranous findings on LM (diffuse and global membranous spike or bubbling with IgG granular deposition) (LM group; n = 30) and those on IF (nothing morphological changes but diffuse and global IgG granular deposition) (IF group; n = 13). CRR was defined as a spot urine protein/creatinine ratio &lt;0.5 g/g Cr and normal serum Cr or an increase in serum Cr within 10% of the pretreatment value. <h3>Results:</h3> The mean age was 41.1 ± 14.6 years (male: female = 9:34), disease duration 6.9 ± 7.1 years, urine protein/Cr ratio 4.1 ± 3.1 g/Cr, serum Cr 0.96 ± 0.43 mg/dl. After three years, 69.2% of cases in the IF group achieved CRR and had no ESRD, while those in the LM group were lower at 30.0% (p=0.0163), and five patients (16.7%) developed ESRD (p=0.0489). <h3>Conclusion:</h3> The mixed-type LN, as defined by LM, had a significantly poor prognosis than the one determined only by IF. <h3>REFERENCES:</h3> [1] Weening JJ, et al. Kidney Int. 2004;65(2):521-30. [2] Bajema IM, et al. Kidney Int. 2018;93(4):789-796. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0054 PREDICTIVE ROLE OF CENTRAL SENSITIZATION IN KINESIOPHOBIA AND EXERCISE CAPACITY IN BEHCET DISEASE
Authors: G. Alp, F. Sari
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Behcet's disease (BD) may experience heightened pain sensitivity, potentially related to central sensitization (CS), especially if the nervous system is involved in the disease process. <h3>Objectives:</h3> This study aims to determine the frequency of CS in BD patients and to evaluate the relation of CS with fear of falling, exercise capasity, disease activity, and quality of life. To evaluate the correlations of these parameters with the CS score. <h3>Methods:</h3> Forty-eight patients with BD were included in the study between September 2023 and December 2023. Tampa Kinesiophobia Scale(TKS), six-minute walk test (6MWT), and the central sensitization inventory short form was administered to all participants. The activity of BD was determined by the Behçet's disease current activity form (BDCAF). <h3>Results:</h3> CS was detected in 59.6% of patients with BD. Of the 22 of 48 patients (45.8%) were male, the mean patient age was 41.6±11.2, and the median disease duration was 8 (10) years. In their current treatments, 35 of them (72.9%) were using colchicine, and 20 of them (41.7%) were using azatiopurine. In patients with CS, fear of exercise was higher, quality of life was poor, and disease activity was significantly higher. These patients had higher 6MWT pre- and post-test muscle fatigue scores and shorter walking distances (Table 1). There were high correlations between the CS scores and the TKS, 6MWT, and BDCAF scores in patients with BD (Rho = 0.526; <i>p</i> &lt;.001, Rho = 0.474; <i>p</i>.001, and Rho = 0.560; <i>p</i>&lt;.001, respectively) Table 2. <h3>Conclusion:</h3> CS is associated with kinesiophobia, decreased exercise capacity, and higher disease activity in Behcet's patients. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>