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Lymphocytic infiltration of the salivary and lachrymal glands is a hallmark of primary Sjögren's Syndrome (PSS). Chronic inflammation leads to progressive destruction of the glands resulting in dryness of the eyes and mouth termed sicca. The course and pace of sicca development in PSS is elusive. To explore the effect of symptom duration on sicca and disease activity. Data of patients from the PSS registry of the Medical University of Graz fulfilling the 2016 ACR/EULAR classification criteria for PSS were analysed. We analysed disease duration and total duration of sicca symptoms, Schirmer's test, unstimulated salivary flow test (USF), stimulated salivary flow test (SSF) and ESSDAI (EULAR Sjögren's Syndrome disease activity index), Evaluators Global Assessment (EGA), and focus score of the initial lip salivary gland biopsy. In addition, salivary gland ultrasound (SGUS) was performed using the De Vita scoring system. Free light chains (FLC) ĸ and λ, IgG, rheumatoid factor (RF) IgA were determined. The patient's perspective was analysed by ESSPRI and PSS-QoL. Data from 159 PSS patients were analysed, of whom 92.6% (n=148) were female. The mean age was 59.3±12.2 years (± standard deviation), with a mean disease duration of 5.5±5.4 years and a mean sicca duration of 11.6±7.4 years. Interestingly, results of USF, SSF, and Schirmer's Test did not decrease with duration of disease or sicca. The lack of correlation was probably caused by a subset of patients in whom glandular function remained stable 10 to 33 years after the onset of sicca. We defined this subgroup of patients by an SSF >0.5ml/min and with a symptom duration >10 years (SFSD10). 19.5% (n=31) of our PSS patient were categorised into this group. SFSD10 showed a significantly higher Schirmers test (median 0 (0-35] vs 3[3-35], p<0.01), lower ESSPRI-dryness (7 [1-10] vs. 5[1-10], p<0.05), PSS-QoL dryness (11[2-22] vs. 8 [3-15], p<0.01), lower IgG (15.5 [7.4-38.1] vs. 11.2 [6.5-30.5], p<0.05) and FLC-K (25 [3.9-134] vs. 20.2 [10.3-63.6], p<0.05) compared to non-SFSD10-group. SGUS De Vita score was significantly lower in the SFSD10 subgroup (median 1 [0-2] vs. 2 [1-3], p=0.045). We found no difference in ESSDAI, focus score, antibody status, pain, fatigue or HRQL between the two groups. Our results indicate the presence of a subgroup of PSS patients with preserved glandular function despite long-standing disease. NIL. NIL. None Declared.

Background:Giant cell arteritis (GCA) is an immune-mediated granulomatous vasculitis affecting large and medium-sized vessels. Vascular ultrasound, the diagnostic gold standard, has limitations in evaluating the aorta. Therefore, positron emission tomography-computer tomography (PET-CT) with [18F]FDG has emerged as a diagnostic alternative. However, interpretation can be challenging as glucose metabolism also occurs in vascular remodeling. The novel radiotracer [68Ga]Ga-DOTA-Siglec-9 could improve PET-CT diagnostic capabilities. Early studies in animals and humans have validated its safety, tolerability, and potential efficacy in identifying inflammation. Siglec-9 is the leukocyte ligand for vascular adhesion protein 1 (VAP-1). Under physiological conditions, VAP-1 resides in intracellular vesicles within various cell types, including endothelial cells. Inflammatory stimuli prompt its translocation to the endothelial cell surface, enabling immune cell adhesion and migration. This upregulation of VAP-1 during inflammation renders [68Ga]Ga-DOTA-Siglec-9 PET-CT particularly interesting for GCA.Objectives:Evaluating [68Ga]Ga-DOTA-Siglec-9 PET-CT for Disease Activity Detection in Giant Cell Arteritis.Methods:We recruited a patient with recurrent GCA disease activity. The patient underwent a [68Ga]Ga-DOTA-Siglec-9 PET-CT scan with an injection of 120 MBq [68Ga]Ga-DOTA-Siglec-9. Fifty-one minutes post-injection, we conducted a low-dose CT for attenuation correction and a whole-body PET scan (one minute/ bed). We also used standard imaging methods, such as vascular ultrasound for the temporal, facial, axillary, carotid, and vertebral arteries, along with aortal MRI and routine laboratory tests.Results:A 90-year-old male patient with GCA, diagnosed in 2018, was enrolled. The patient reported recurrent GCA symptoms, including bitemporal headaches and night sweat. At the time of scan, he received methotrexate 15 mg per week and a daily dose of 2 mg prednisolone. His C-reactive protein level was elevated at 21 mg/l. [68Ga]Ga-DOTA-Siglec-9 PET scan revealed increased tracer uptake (SUVmax) in the subclavian artery (2.5), aortic arch (2.9), and heart (2.9), in contrast to an uptake of 1.5 in the liver (Figure 1). Notably, the increased uptake in the descending aorta (3.5) abruptly diminished to 2.2 when passing the diaphragm, with no changes in vessel caliber observed in CT (Figure 1). Vascular ultrasound and MRI did not reveal any pathological findings (data not shown). The injection of [68Ga]Ga-DOTA-Siglec-9 was well tolerated.Figure 1.Enhanced [68Ga]Ga-DOTA-Siglec-9 uptake in aortic and subclavian regions of a giant cell arteritis patient in positron emission tomography imagingWhole-body PET images of GCA patient (male, 90 years old) after intravenous injection of 120 MBq of [68Ga]Ga-DOTA-Siglec-9. Distribution of [68Ga]Ga-DOTA-Siglec-9 51 min after injection, based on imaging for one minute per bed position, revealed increased uptake in projection on the aortic and subclavian vessels and the heart. The increased uptake in the descending aorta diminishes abruptly while passing the diaphragm, without any caliber jump in the CT images.Conclusion:This study presents the first application of [68Ga]Ga-DOTA-Siglec-9 PET-CT in a GCA patient. PET-CT imaging demonstrated increased tracer uptake in the subclavian artery and aortic arch, with a localized and abrupt reduction. Notably, there were no corresponding findings in conventional imaging. We hypothesize that [68Ga]Ga-DOTA-Siglec-9 PET-CT might offer unique potential for precise, localized mapping of affected vascular tissue in GCA, especially during relapse. This could revolutionize the current diagnostic approach, leading to more targeted strategies for monitoring disease activity. Given these encouraging findings, larger scale studies are essential to fully elucidate the potential role of [68Ga]Ga-DOTA-Siglec-9 PET-CT in the diagnostic landscape of GCA.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Juvenile localised scleroderma (jLS) is a rare rheumatic disease in children characterized by inflammation and fibrosis in the skin [1, 2]. The cause and pathogenesis of jLS remain unclear, and both skin lesions and possible extracutaneous involvement may result in functional impairment and growth disturbances [2]. The treatment options to cure jLS are limited [3].In recent years, omics technologies have been used to identify novel biomarkers in different diseases [4, 5]. Among the different omics technologies, metabolomics and lipidomics provide snapshots of the metabolic network.Objectives:We aim to identify biomarkers and treatment targets for jLS using metabolomics and lipidomics.Methods:Children with jLS and age-matched controls were recruited at Bambino Gesù Children’s Hospital, Roma, Italy. The characteristics of the participants are shown in Table 1.Plasma samples from 9 controls and 12 patients with jLS (before treatment initiation and 17 months after treatment) were sent to Swedish Metabolomics Center, where liquid chromatography–mass spectrometry and gas chromatography–mass spectrometry were performed (Figure 1A). Peak intensities were recorded, and the data analysis was performed using Metaboanalyst 5.0 and Graphpad Prism 10 software. Pathway enrichment bubble plots were generated using SRplot [6]. Mann-Whitney test was used to compare healthy control and baseline patient groups, and Wilcoxon test was used to compare differences between baseline and treated patients.Results:In total, 250 metabolites and 194 putative lipids were annotated (Figure 1A). Patients at baseline had significantly lower peak intensities of lenticin, 3-hydroxybutyrylcarnitine, 1-dodecanoyllysophosphatidylcholine, phosphatidylcholine (PC) 38:6 and 40:9, and phosphatidylserine (PS) 38:1 as well as significantly higher peak intensities of L-tyrosine, phenylpyruvic acid, (3-hydroxyphenyl)hydracrylate, and cortisol compared to controls (Figure 2B). After treatment, peak intensities of adenosine monophosphate, hypoxanthine, 3-phosphoglyceric acid, lysophosphatidylcholine (LPC) 18:2, Cholesteryl Octanoate (CE 8:0), and 2-Hydroxylauroylcarnitine (CAR 12:0) were decreased, whereas peak intensities of L-octanoylcarnitine and eleven molecular species of triacylglycerols were increased compared to baseline patients (Figure 2D). The top enriched pathways are shown in Figure 2C and 2E.Conclusion:We have described the metabolic profile in blood of children with jLS for the first time. Children with jLS show a distinct metabolic profile compared to healthy children, especially in tyrosine-related pathways. Compared to baseline levels, the metabolism of several amino acids was altered after treatment, and the energy storage function might be modified as eleven molecular species of triacylglycerols were found decreased.REFERENCES:[1] Zulian, F., et al., Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis, 2019. 78(8): p. 1019-1024.[2] Li, S.C. and R.J. Zheng, Overview of Juvenile localized scleroderma and its management. World J Pediatr, 2020. 16(1): p. 5-18.[3] Li, S.C., Treatment of juvenile localized scleroderma: current recommendations, response factors, and potential alternative treatments. Current Opinion in Rheumatology, 2022. 34(5): p. 245-254.[4] Puentes-Osorio, Y., et al., Potential clinical biomarkers in rheumatoid arthritis with an omic approach. Autoimmunity Highlights, 2021. 12(1).[5] Xiao, Y.A., et al., Multi-omics approaches for biomarker discovery in early ovarian cancer diagnosis. Ebiomedicine, 2022. 79.[6] Tang, D., et al., SRplot: A free online platform for data visualization and graphing. PLoS One, 2023. 18(11): p. e0294236.Table 1.Characteristics of the participantsCharacteristicsPatientsControlsN129Age, years10±410±4Females, n (%)6 (50)8 (89)Follow-up, months17±3Treatment:Prednisolone, n (%)10 (83)Methotrexate, n (%)12 (100)Tocilizumab, n (%)2 (17)Figure 1.Study design (A), volcano plot of all the annotated metabolites and lipids (B, D), metabolic pathway analysis with the most changed metabolites (C, E).Acknowledgements:Participants who donated the blood samplesDisclosure of Interests:Yuan Zhang: None declared, Angela Aquilani: None declared, Rebecca Nicolai: None declared, Fabrizio De Benedetti Speaker for Novartis and SOBI, Emiliano Marasco: None declared, Cristina Maglio: None declared.

Despite intensive lipid-lowering interventions, patients treated with statins develop atherosclerotic cardiovascular disease (ASCVD), and these patients have an increased risk of developing recurrent cardiovascular events during follow-up. Therefore, there is a need to focus on the residual risks in patients in statin therapy to further reduce ASCVD. The aim of this study was to retrospectively investigate the 10-year trend (2011–2019) regarding changes in polyunsaturated fatty acids (PUFAs) in patients with acute coronary syndrome (ACS) in a single center. We included 686 men and 203 women with ACS admitted to Kagawa Prefectural Central Hospital. Plasma PUFAs, including eicosapentaenoic acid (EPA), docosahexaenoic acid (DHA), arachidonic acid (AA), and dihomo-γ-linolenic acid (DGLA), were measured at admission for suspected ACS. A secular decreasing trend in the levels of EPA and DHA and the EPA/AA ratio, but not of AA and DGLA, was observed. The analyses based on age (>70 or <70 years) and sex showed that the decreasing trend in the levels of EPA and DHA did not depend on age and remained significant only in men. Further studies are needed to obtain robust evidence to justify that the administration of n-3 PUFA contributes to the secondary prevention of ACS.

Background:Interstitial lung disease (ILD) is the foremost contributor to mortality in systemic sclerosis (SSc). High-resolution computed tomography (HRCT) remains the gold standard for ILD diagnosis. While previously recommended for all patients at the time of SSc diagnosis, HRCT screening is now conditionally advised by the 2023 ACR guideline for managing ILD across rheumatic diseases, explicitly targeting patients at increased risk of developing ILD. The impact of not screening all SSc patients for ILD has so far not been assessed in detail.Objectives:To explore the impact of HRCT screening on ILD identification, ILD progression, and survival across two countries with differing screening approaches.Methods:This multicenter study involved 314 Romanian SSc (Ro-SSc) patients from “Sfanta Maria” Clinical Hospital in Bucharest (with non-universal HRCT-ILD screening) and 905 Norwegian SSc (Nor-SSc) patients from Oslo University Hospital (with routine HRCT screening since 2000). The study aimed to assess the prevalence of ILD and factors associated with HRCT conduction over three periods: 2000-2010, 2010-2020, and 2020-2023. Baseline and last available follow-up pulmonary function tests (PFTs) were retrieved. ILD progression was categorized based on forced vital capacity (FVC) decline as severe (>10% FVC decline) and moderate (5-10% FVC decline). We defined the presence of significant progression in the absence of HRCT as likely ILD. Logistic and Cox regression were conducted.Results:Baseline screening for ILD with HRCT was performed in 37% (117) Ro- and 96% (871) Nor-SSc patients. The use of HRCT increased in the Ro-SSc cohort over time, with 60% of all patients undergoing HRCT after 2020, while remaining stable in the Nor-SSc cohort. HRCT screening in the Nor-SSc cohort was not based on risk factors for ILD. In the Ro-SSc cohort, HRCT screening was significantly associated with SSc-ILD risk factors, including male sex, diffuse cutaneous SSc, anti-topoisomerase I positivity, presence of respiratory symptoms, and lower FVC (Figure 1). Among all patients who underwent HRCT, 95% (190) of Ro- and 45% (388) of Nor-SSc were diagnosed with ILD. At the time of ILD diagnosis, Ro-SSc patients showed longer disease duration (4.41±3.2 vs. 2.1±2.9 years, p<.001) and more severe ILD with lower FVC (74.4±17.4% vs 90.4±23.7, p<001), and a higher prevalence of ≥20% fibrosis on HRCT (40% vs 11%, p<.001), compared to the Nor-SSc cohort. To estimate the impact of only screening SSc patients with risk factors for ILD, we next assessed patients in the Ro-SSc cohort not screened for ILD by HRCT and compared them to patients screened with HRCT and Nor-SSc patients without HRCT. Among the 40% of Ro-SSc cases without HRCT, a significant number experienced FVC decline, while Nor-SSc patients without HRCT showed no decline (Figure 2). The likelihood of ILD in Ro-SSc patients without HRCT was confirmed through logistic regression [OR 1.95 (0.49-2.63), p<0.001)]. In total, 23% of Ro-SSc and 30% of Nor-SSc patients died. As expected, in both HRCT-assessed SSc cohorts, FVC decline was predictive for mortality (Ro-SSc: HR 4.32, 95%CI 0.21-6.58, p<.001; Nor-SSc: HR 2.05, 95% CI 0.42-10.0, p<.001). In Ro-SSc patients without HRCT, FVC decline was predictive for mortality in the same range (HR 4.72, 95 % CI 1.43-15.5, p=0.01).Conclusion:Our results suggest that upfront HRCT screening of all SSc patients allows for an earlier diagnosis of ILD. Selective HRCT referral practices based on known risk factors leave a significant number of progressive ILD cases undiagnosed, with missed opportunities for treatment. Overall, the current results suggest that referring all newly diagnosed SSc patients to primary ILD screening by HRCT is warranted.REFERENCES: NIL. Acknowledgements:NIL.Disclosure of Interests:Cristina Nita: None declared, Laura Maria Groșeanu: None declared, Daniela Opriș-Belinski: None declared, Mihaela Popescu: None declared, Athir Eddan: None declared, Emily Langball: None declared, Håvard Fretheim Boehringer Ingelheim, Bayer, Henriette Didriksen: None declared, Hilde Jenssen Bjørkekjær Jannsen, Phuong Phuong Diep: None declared, Torhild Garen: None declared, Mike Durheim: None declared, Øyvind Midtvedt: None declared, Trond M Aaløkken: None declared, Oliver Distler Boehringer Ingelheim, Janssen, Medscape, CITUS AG, 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Argenx, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Orion, Prometheus, Redxpharma, Roivant, Topadur and UCB, Øyvind Molberg: None declared, Andra Bălănescu: None declared, Anna-Maria Hoffmann-Vold Boehringer Ingelheim, Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Novartis and Roche, ARXX, BMS, Boehringer Ingelheim, Genentech, Janssen, Medscape, Merck Sharp & Dohme and Roche, Boehringer Ingelheim, Janssen.

Background:Rheumatoid arthritis (RA) is a chronic autoimmune disease caused by genetic and environmental factors characterized by joint inflammation. The JAK-STAT inhibitors (jakinibs) are among the therapeutic options together with conventional and biological Disease Modifying Antirheumatic Drugs (DMARDs) [1].Objectives:To determine the impact of jakinibs in monocyte and Natural Killer cells (NK) subsets in RA patients by flow cytometry.Methods:A total of 51 patients treated with jakinibs were recruited, 20 healthy donors and 20 RA patients treated with biological DMARDs, both paired by sex and age with the jakinibs group, were enrolled as controls. Peripheral blood mononuclear cells (PBMCs) were isolated by density gradient using Ficoll. Multiparametric flow cytometry was performed for immunophenotype characterization of different subsets of NK cells acquired in DX Flex cytometer (Beckman Coulter).Results:Within the jakinib group, 22 (43.14%) patients were treated with baricitinib (Bari), (27.45%) 14 with tofacitinib (Tofa), 11 (21.57%) with filgotinib (Filgo) and 4 (7.84%) with upadacitinib (Upa); whereas in the RA control group: 11 patients were treated with tocilizumab and 9 treated with abatacept. A significant decrease in the percentage of cytotoxic NK Dim (CD56+CD16+) subset was observed in the jakinib group in comparison to the RA control group (92.18 (89.18-96.59) vs 87.28 (81.29-90.93) p=0.001). There were significant differences between jakinib group and both healthy and RA control groups in the percentage of activated NK Dim expressing Nkp30 (59.51 (32.19-80.34), 89.74 (84.81-95.81) and 85.28 (67.08-93.16), respectively; p values: <0.0001 and 0.007). In addition, the percentage of intermediate monocytes (CD14+, CD16+) was decreased in jakinib group in comparison with RA and healthy controls (9.84 (5.12-16.36), 19.41 (14.39-30.30) and 20.55 (15.34-30.09), respectively; p values: 0.001 and <0.0001).Conclusion:The JAK-STAT inhibition by jakinibs affects innate cells in a different way than biological DMARDs do. The decrease in both the cytotoxic activated NK subset and intermediate monocytes could explain some of the side effects caused by these drugs related to intracellular threats such as viral infections or possible neoplasia appearances. Further functional studies should be addressed to better understand the impact of jakinibs in innate cell subsets and their relationship with adverse effects in RA patients.REFERENCES:[1] Meudec L, Richebé P, Pascaud J, Mariette X, Nocturne G. Janus kinase inhibitors alter NK cell phenotypes and inhibit their antitumour capacity. Rheumatology (Oxford). 2023 Aug 1;62(8):2855-2863. DOI: 10.1093/rheumatology/keac710. PMID: 36583542.Figure 1.Dotplots depicting the percentage of NK Dim cells (a), NK Dim Nkp30 cells (b) and Intermediate Monocytes (c) in the three groups studied (healthy controls in green dots, RA controls in grey squares and jakinib patients in beige rhombuses).The whiskers represent the median, first and third quartile. P values are represented as follows: * P <.05, ** P <.01, ***P <.001, ***P<.0001. NS: not significant differences found.Acknowledgements:NIL.Disclosure of Interests:Juan José Fernández Cabero: None declared, Carmen Lasa-Teja: None declared, Alejandra Comins-Boo: None declared, David San Segundo: None declared, Marcos López Hoyos: None declared, Ricardo Blanco Pfizer, Roche, Bristol-Myers, Lilly, Galapagos, Novartis, Janssen, GSK, and MSD.

The use of diagnostic laboratory tests is increasing worldwide. Improper test utilization (ITU) is a common problem for all healthcare systems as it costs substantial expenses for the health systems and impacts optimal patient care. The present small-scale survey aims to highlight the current practice of ITU among the labs and physicians, and investigate the actions of diagnostic laboratories towards ITU, and identify the reasons affecting test ordering decisions among physicians. A cross sectional study based on two different surveys was developed and distributed from March 2017 to April 2017 to laboratory supervisors and physicians (clinicians) at Hamad Medical Corporation (HMC), Qatar. Fourteen laboratory supervisors and eighty-nine physicians were surveyed about improper test utilization practices. The overall results are descriptive data. The overall proportion of improperly utilized tests detected by the laboratory supervisors were 50.0%, 35.7%, and 14.3% for overused, misused, and underused lab tests, respectively. Among the physicians, 91% used the electronic ordering template to select the appropriate tests. Moreover, 78.7% of the physicians used the clinical guidelines, while 73% were not employing the laboratory handbook. Furthermore, 95.5%% of the physicians preferred to get feedback about inappropriate tests, while 51.1% were not receiving any, and 40.9% were rarely receiving. Finally, 67.4% were unaware of the tests' costs among surveyed physicians, and 63.6% showed a willingness to reduce their orders if the cost was high and unnecessary. The physician's and the laboratories' communication were inadequate and not systematized, causing ITU practices. The improvement strategy should focus on the communication between clinical labs and physicians and enhance physician implementation to order appropriate lab tests. This could be achieved by conducting legitimate educational methodologies, continuous feedback reviews, ongoing audits, executing health information technology instruments, engaging laboratory practice guidelines, and applying demand management and testing algorithms.

Background:The use and utility of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) increased over the years, due to correlations between these autoantibodies, specific clinical manifestations, response to treatment, and prognosis V[1,2]. Studies on the prevalence of MSAs/MAAs and their association with the clinic often differ, probably due to inclusion criteria, demographics, and screening assays used [3]. There is limited information about the prevalence of these autoantibodies in the Hispanic population [2]. Understanding the prevalence and frequency in different patient groups contributes to the standardization and rapid adoption of antibodies in the evaluation of inflammatory myopathies in these groups.Objectives:The aim is to describe the prevalence of autoantibodies for idiopathic inflammatory myopathies (IIM) in Hispanic patients.Methods:A cross-sectional study was performed from July 2016 to December 2023 in the rheumatology service of the University Hospital “Dr. José Eleuterio González”, Mexico. An immunoblot panel of autoimmune inflammatory myopathies with 17 austoantibodies was performed on patients with suspected IIM. The results were compiled in a database during this period. The cut-off points for categorizing the immunoblot results were: 0 - 7 negative; 8 - 14 borderline; 15 - 35 weak positive; 36-70 moderate positive; and 71-255 strong positive.Results:A total of 997 patients were screened in the last 7 years, in which 446 (44.7%) patients reported a moderate and strong positive result. The highest autoantibody seen with 129 (12.9%) patients was RO52 autoantibody. In contrast, the autoantibodies with the lowest prevalence for moderate and strong positive results were OJ with 5 (0.5%), EJ with 7 (0.7%), and SAE1 with 15 (1.5%). Among the autoantibodies specific for dermatomyositis, the highest prevalences were Mi-2beta with 36 (3.6%) and Mi-2alpha with 33 (3.3%). Figure 1 shows the rest of the autoantibodies.Figure 1.Prevalence of autoantibodies for inflammatory idiopathic myositis in Hispanic patients.Conclusion:This study demonstrates the prevalence of autoantibodies with suspected IIM, presenting relevant information on the distribution of these markers in the population studied. RO52 autoantibody was the most prevalent in Hispanic patients, at least 3 times higher than any other autoantibody. Those with the lowest prevalence were EJ and OJ. The specific autoantibodies to dermatomyositis with the highest prevalence were Mi-2beta and Mi-2alpha, whereas SAE was the lowest.REFERENCES:[1] Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med. 2016;280(1):8-23.[2] Gracia-Arechiga TS, Villarreal-Alarcon MA, Hermosillo-Villafranca J, Pérez-Onofre I, et al. Seventeen myositis autoantibodies: serological profile of Hispanic patients with idiopathic inflammatory myopathies. Ann Rheum Dis. 2018; 77:1537.[3] Hodgkinson LM, Wu TT, Fiorentino DF. Dermatomyositis autoantibodies: how can we maximize utility? Ann Transl Med. 2021;9(5):433.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Announcing a diagnosis in rheumatology holds crucial importance for patients with chronic rheumatic diseases. Unlike other medical fields like oncology, little is known about rheumatology announcement practices from the patients’ perspective, and how well these protocols meet their needs and expectations.Objectives:The aim of this study is to investigate and evaluate the communication practices surrounding diagnostic announcements in rheumatology from the perspective of patients.Methods:A descriptive cross-sectional study was conducted involving patients with various chronic rheumatic diseases, including rheumatoid arthritis (RA), spondylarthritis (SpA), psoriatic arthritis (PsA), or undifferentiated rheumatic disease.Sociodemographic data, disease-related information, and details about the diagnosis announcement process were collected through a questionnaire developed collaboratively by two rheumatologists.Results:In total, 50 patients were included, with a mean age of 52.4 ± 14.8 years [28 - 77] and a sex ratio of 0.28. The average age at the time of diagnosis was 43.8 ± 15.9 years [18 - 75]. Diagnoses were distributed as follows: 25 (50%) with RA, 15 (30%) with SpA, 4 (8%) with PsA, and 2 with undifferentiated inflammatory disease. The average disease duration was 8.5 ± 6.5 years [1 - 35]. Nine patients (20%) had unfavorable socio-economic conditions, 36 (78%) were married, and in terms of education, 5 patients were illiterate, 17 (34%) had primary education, while 7 (14%) had university degrees. Fourteen patients (28%) used a non-specific Tunisian dialect term to refer to their disease, 19 (38%) described it as an inflammatory joint disease without seeking further specifics, and 15 patients (30%) knew the scientific name of their disease in Arabic or French. All patients remembered the day of the diagnosis announcement. Twenty-seven patients (54%) indicated that their treating physician was the one who made the diagnosis announcement. Sixteen (32%) patients believed their doctor used simple language, while 34 (68%) preferred simpler language. The majority of patients (63%) felt a lack of empathy from the doctor, with 32 (64%) wishing for more empathy. Additionally, 32 patients (64%) found the allocated time insufficient, and 36 (72%) requested more time. In 58% of cases, the announcement took place at the patient’s bedside, but patients’ preference was for an announcement in the doctor’s quiet office. Regarding the information provided, 32 patients (64%) reported that not all information about their disease (vital and functional prognosis, complications, repercussions) was communicated during the announcement, and only 6 preferred a gradual release of information over multiple consultations. Finally, during this announcement, 40 patients (80%) were alone, of which 12 (24%) would have preferred to be accompanied.Conclusion:This investigation highlights several problems in diagnosis disclosure practices, emphasizing the need for improved communication of essential information and a more empathetic approach, particularly for patients in potential denial.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Anti-synthetase syndrome constitutes a dynamically evolving subset of Idiopathic Inflammatory Myositis, marked by continual discoveries of novel antibodies, clinical parameters and treatment modalities. However, the nomenclature and appropriate abbreviations for this syndrome are plagued by heterogeneity and ambiguity, leading lack of consistency in literature.Objectives:The aim of this study is to evaluate existing diversity in disease names and abbreviations within the current literature, with a future goal to develop consensus on the nomenclature.Methods:A comprehensive search of PUBMED was conducted from January 1, 1984 (the initial description of anti-synthetase autoantibodies) to November 30, 2023, encompassing all pertinent articles published within this timeframe. The search terms employed were “antisynthetase syndrome,” “anti synthetase syndrome,” and “anti-synthetase syndrome.” Inclusion criteria comprised all articles published in English, excluding animal studies and those solely focused on anti-synthetase autoantibodies rather than the syndrome itself. The articles were then screened for presence of terminology used for anti-synthetase syndrome and any abbreviations for the disease.Results:The search yielded a total of n=936 items with the specified terms. After excluding 303 irrelevant articles and 58 non-English publications, the remaining n=575 articles underwent detailed review of the abstract and the full article. The data collected was representative of 47 nationalities from all over the globe. The term “anti-synthetase syndrome” made its inaugural appearance in 1992, and for the subsequent decade, no abbreviations were employed (until 2002) (Figure 1). Out of n=575, there was variability in the terminology used as well with 54.7% (n=314) using ‘antisynthetase syndrome’ and 43.4% (n=249) preferring ‘anti-synthetase syndrome’ with few articles mentioning novel names as well (Table 1). Among these, 394 articles used abbreviations while 181 did not. Most utilized term was ASS (44.3%, n=255), followed by AS (8.2%, n=47); ASSD (6.8%, n= 39) and ASyS (5.2%, n=30). Other less commonly deployed abbreviations included ARS (1.4%), anti-SS (1.2%), ASA (0.5%), Anti-SS-OM (0.3%), Anti-ARS (0.2%), SynS (0.2%) and Anti ARS syndrome (0.2%) (Table 1).Conclusion:A conspicuous discordance in nomenclature is evident, with about half using antisynthetase syndrome and other half using anti-synthetase syndrome. Moreover, significant heterogeneity exists in abbreviations with most employing ASS. There is a pressing need to bridge this disparity among the various terminology as well as abbreviation to establish a uniform identifier for the disease. Therefore, it is imperative to cultivate a consensus on an all-acceptable nomenclature to alleviate confusion and enhance clarity in communication.REFERENCES:[1] Santiago Villalobos R, López-Campos Bodineau JL, Rodríguez Becerra E, Laserna Martínez E, Luque Crespo E, Borja Urbano G. Síndrome antisintetasa y afección pulmonar intersticial. Descripción de 6 casos [Antisynthetase syndrome and interstitial lung involvement. Report of 6 cases]. Arch Bronconeumol. 2002 Oct;38(10):495-8. Spanish. doi: 10.1016/s0300-2896(02)75273-3. PMID: 12372202.[2] Sturgess A. Recently characterised autoantibodies and their clinical significance. Aust N Z J Med. 1992 Jun;22(3):279-89. doi: 10.1111/j.1445-5994.1992.tb02126.x. PMID: 1497555.[3] Mahler M, Miller FW, Fritzler MJ. Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. Autoimmun Rev. 2014 Apr-May;13(4-5):367-71. doi: 10.1016/j.autrev.2014.01.022. Epub 2014 Jan 11. PMID: 24424190; PMCID: PMC3970575Acknowledgements:NIL.Disclosure of Interests:Anushka Aggarwal: None declared, Tanya Chandra: None declared, Parth Ladha: None declared, Srijan Mittal: None declared, Saloni Haldule: None declared, Simran Nirmal: None declared, Namratha Edpuganti: None declared, Nakul Jain: None declared, Rohit Aggarwal Actigraph, Alexion, ANI Parmaceuticals, Argenx, AstrZeneca, Boehringer-Ingelheim, Bristol Myers-Sqibb, CabalettaBio, Capella Bioscience, Corbus, CSL Behring, EMD Serono, Galapagos, Horizon Therapeutics, I-cell, Janssen, Kezar, Kyverna, Merck, Novartis, Nuvig Therapeutics, Octapharma, Pfizer, Regeneron, Roivant, Sanofi, Teva, Artsome, Capstanx, Manta, Boehringer Ingelheim, Bristol Myers-Squibb, EMD Serono, Jassen, Mallinckrodt, Pfizer, Q32.