Anti-ribonucleoprotein (RNP) (auto)antibodies are frequently detected in patients with systemic lupus erythematosus (SLE) although their associations with SLE disease activity remain incompletely understood. We compared patient characteristics, including disease severity outcomes, between adult patients with SLE with or without anti-RNP antibodies (RNP + versus RNP-). We used data from a national, multicentre registry of SLE patients in Australia, collected prospectively between 2007 and 2023. Anti-RNP status and other serological profiles along with demographics were recorded at enrolment. At each routine clinic visit, medication details and disease activity indicators (SLEDAI-2 K, PGA) were captured, and treat-to-target (T2T) states, lupus low disease activity state (LLDAS) and DORIS remission (REM), were determined. Clinical characteristics were compared between RNP + and RNP- patients at 6 months (182 ± 30 days) and 12 months (365 ± 30 days) from enrolment. A total of 587 patients were studied. 174 (29.6

Idiopathic multicentric Castleman disease (iMCD) is a rare condition. The pathogenesis is incompletely understood; however, interleukin-6 (IL-6) is a major mediator. The clinical presentation is heterogeneous, from mild constitutional symptoms to severe multi-organ failure. The diagnosis is challenging, as it incorporates clinicopathologic criteria and requires careful evaluation to exclude various systemic disorders. Targeting IL-6 activity forms the cornerstone of modern therapy for iMCD, with siltuximab recommended as first-line therapy. Rituximab-based regimens are recommended for second-line therapy. However, many patients do not achieve adequate responses with limited evidence to guide further therapy. In the context of these substantial challenges, herein we provide a multidisciplinary Australasian clinical practice guideline to characterise clinical and pathological features, summarise treatment pathways and discuss clinical outcomes of the condition. The objective is to develop a multidisciplinary clinical practice guideline in the diagnosis and management of iMCD in Australia.

BACKGROUND:Sjögren's Disease (SjD) is a systemic autoimmune disease that is associated with a significant reduction in health-related quality of life (HRQoL). The goal of this study was to better understand HRQoL using a multi-national and rigorously defined SjD population according to 2016 ACR/EULAR classification criteria. METHODS:The Outcome Measures in Rheumatology Clinical Trials (OMERACT) Sjögren's Disease Working Group conducted three regionally based focus groups (United States, Australia/China, the Netherlands) for adult patients with SjD who met classification criteria. The focus groups were semi-structured and designed to elicit feedback regarding important life impact domains, or aspects of disease, that should be included in future research. Additionally, a brief demographic survey was administered. Focus group transcripts were coded and analyzed for theme saturation and interpretation. RESULTS:There were 29 participants with SjD who completed the focus groups (25 females, 4 males); 28 participants completed the surveys. Five codes and 19 subcodes were identified corresponding to the following relevant domains: pain interference, physical activity, dryness, fatigue, oral health, autonomic function, and sequelae of inflammation, brain fog, pregnancy/fertility, emotional health, social participation and engagement with others including medical providers, and worker productivity. CONCLUSION:These findings from a multi-national sample reaffirm the relevance of established symptoms such as pain, fatigue, and dryness while elucidating a broader spectrum of disease impact reported by individuals with SjD. Domains related to cognitive impairment, autonomic dysfunction, oral health, emotional well-being, and social and occupational functioning emerged as salient features of the patient experience. These data support the need to expand current outcome frameworks to better capture the multidimensional burden of SjD and inform the development of more comprehensive, patient-centered assessment tools.

Objective Childhood-onset SLE (cSLE) is often described as more severe than adult-onset disease (aSLE) based largely on cross-sectional studies. We examined differences in disease characteristics, medication use and long-term outcomes between cSLE and patients with aSLE in adulthood using longitudinal data from a national cohort to address this knowledge gap. Methods A retrospective cohort study was conducted using the Australian Lupus Registry and Biobank. Data included demographics, disease duration, autoantibody profile, classification criteria, time-adjusted mean SLE Disease Activity Index 2000 (SLEDAI-2K AMS), SLE Damage Index (SDI) and SF36 health-related quality of life data. Key outcomes were AMS and time in the Lupus Low Disease Activity State (LLDAS) and damage accrual. Bivariate tests were used for group comparisons. Results Of 519 patients with SLE enrolled between 2011 and 2022 with ≥12 months of data available, 68 (13%) had cSLE. Median (IQR) age at enrolment was 39 (30–51) years; 88.1% female; majority were of white or Asian ethnicity. At baseline, more patients with cSLE had damage (SDI≥1) (53% vs 40%, p=0·05) and renal involvement (62% vs 37%, p<0·001). Over median (IQR) follow-up of 5 (2·6–9·3) years, patients with cSLE had higher disease activity (median (IQR) AMS 5·0 (3·0–6·4) cSLE vs 3·6 (1·9–5·1) aSLE, p<0.001), were more likely to be in High Disease Activity Status (SLEDAI-2K ≥10 ever) and were less likely to achieve LLDAS-50 (36% vs 51%, p=0·036). Flare rates, damage accrual and quality of life during follow-up were similar between groups. Conclusions Adults with cSLE entered adult follow-up with higher baseline damage and continued to experience a higher longitudinal disease activity burden than patients with aSLE. These findings highlight the importance of early recognition, consistent longitudinal monitoring and timely escalation of therapy during earlier years of disease to reduce long-term disease burden.

OBJECTIVES:Sjögren disease (SjD) is a common systemic autoimmune disease and patients experience a wide range of symptoms with unique emotional, social and physical impacts. Understanding the individual experience of SjD is crucial to providing comprehensive and sensitive care in the clinics. Therefore, the aim of this systematic review was to analyze primary literature that examined the lived experiences of patients with SjD. METHODS:Primary literature qualitatively exploring the lived experiences of SjD patients through interviews and/or focus groups were identified. Papers were included if they were written in English, participants were ≥ 18 years old and they fulfilled a diagnosis of SjD as per the 2002 American-European Consensus or 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria. Thematic analyses were performed using the Thomas and Harden approach. RESULTS:Nine of 1990 screened manuscripts (0.5 %) fulfilled our selection criteria. These comprised a total of 162 participants (154, 95 % female) across 10 countries. Thematic analysis revealed several key themes: the burden of the physical symptoms (such as sicca), social isolation, negative impact on function, unpredictability of the disease, diverse coping strategies, and the challenges of navigating the healthcare system. Few studies addressed any bias in the recruitment of patients or analyses of data. CONCLUSION:SjD patients encounter a large variety of individual experiences in their illness that have important repercussions on quality of life. Understanding these experiences will help create a harmonized set of patient-centered outcomes to inform the generation of Outcome Measurement in Rheumatology (OMERACT) target domains in SjD.

Sjögren disease (SjD) is a prevalent systemic autoimmune condition characterised by exocrine gland dysfunction, systemic inflammation and heterogeneous organ involvement. Current management remains largely symptomatic, with no approved disease-modifying therapies available and substantial unmet clinical need. However, advances in understanding immunopathogenesis have accelerated the development of targeted treatments. Ianalumab, a dual-acting B cell-activating factor receptor (BAFF) receptor inhibitor with B cell-depleting activity, is the first agent to report positive phase 3 results, showing significant improvements in systemic disease activity. Other investigational approaches include BAFF/APRIL pathway inhibition, T-cell-B-cell costimulation inhibition, type I interferon blockade, JAK-STAT, TYK2 and BTK inhibition, neonatal Fc receptor antagonist and endosomal Toll-like receptor inhibition, with exploratory modalities such as RNA-targeted agents and cellular immunotherapy (including Chimeric antigen receptor T therapy) under evaluation for severe or refractory disease. Outcome assessment has also evolved, with European Alliance of Associations for Rheumatology (EULAR) Sjögren Syndrome Disease Activity Index and EULAR Sjögren Syndrome Patient Reported Index providing validated physician- and patient-centred measures. Composite endpoints such as the Composite of Relevant Endpoints for Sjögren Syndromeand the Sjögren Tool for Assessing Response now integrate systemic, symptomatic and functional domains, improving sensitivity and feasibility in trials. Together, these tools support more rigorous evaluation of novel therapies. Overall, therapy in SjD is shifting towards precision, phenotype-informed strategies. Priorities now are confirming long-term safety and durability of response, and demonstrating patient-important benefit. In Australia, the impact of new therapies for SjD will hinge on clear diagnostic pathways, routine use of validated disease activity measures and multidisciplinary care models.

Nomenclature for the disease widely known as Sjögren syndrome has proven unsatisfactory. Patients have perceived ‘syndrome’ as indicative of a vague collection of symptoms, prompting the Sjögren’s Foundation to abandon the term. Furthermore, the traditional distinction between ‘primary’ and ‘secondary’ forms fails to account for the complex interplay between overlapping autoimmune diseases. Following a bibliometric analysis, systematic literature review and a Delphi consensus process with equal involvement of professional and patient representatives, five recommendations are now issued. First, the term ‘Sjögren disease’ should replace ‘Sjögren syndrome’. Second, the acronym ‘SjD’ should be used as an abbreviation for ‘Sjögren disease’. Third, the descriptor ‘associated’ should be used in lieu of ‘secondary’ for Sjögren disease occurring in association with a second systemic autoimmune disease for which classification criteria are fulfilled. Fourth, Sjögren disease is the preferred terminology in common parlance and in clinical diagnosis, without differentiation as to primary and associated forms. Fifth, the differentiation between primary and associated Sjögren is recommended for scientific studies to define a homogeneous population. In conclusion, the consensus endorses ‘Sjögren disease’ as the official nomenclature to acknowledge the distinct pathogenesis of this disorder and to improve clarity in both clinical practice and research. In this Consensus Statement, an international group of experts and patient representatives validates and endorses the transition from the term ‘Sjögren syndrome’ to ‘Sjögren disease’, and issue several additional recommendations regarding the nomenclature of this disorder.

Sjögren's disease (SjD) is a chronic systemic autoimmune disorder characterised by lymphocytic infiltration of the salivary and lacrimal glands, leading to the hallmark symptoms of dry eyes and dry mouth. Beyond glandular dysfunction, many patients experience systemic complications-including B cell hyperactivity, organ-specific inflammation, and a markedly increased risk of non-Hodgkin lymphoma-that are frequently under-recognised and poorly managed. Current treatments remain largely empirical and symptomatic, with limited efficacy in modifying disease progression or restoring immune tolerance. Recent advances have illuminated profound dysregulation in both innate and adaptive immunity, revealing novel therapeutic targets now under investigation in clinical trials, including type I interferon signalling, B cell activation, and co-stimulatory pathways. Central to this dysregulation is T cell-driven pathology: CD8+ T cell cytotoxicity, defective regulatory T cell (Treg) function, and HLA class II-mediated presentation of self-antigens to autoreactive CD4+ T cells are key mechanisms in disease initiation and persistence. A growing body of evidence implicates Ro autoantigens-Ro60 and Ro52-as central targets in SjD pathogenesis. Anti-Ro antibodies are present in approximately 70 % of patients and serve as both diagnostic markers and indicators of systemic involvement. Ro antigens and their corresponding antibodies are consistently detected in inflamed salivary tissues, underscoring their potential as compelling targets for antigen-specific therapy. This review examines the immunopathogenic role of Ro-specific T cell responses in SjD and outlines how engineered Treg-based therapies may enable precise immune modulation, restore tolerance, and provide durable disease control for patients with this complex autoimmune condition.

OBJECTIVES:Sjögren disease (SjD) predominantly affects females, but the early disease presentation in male patients remains poorly characterised due to historically small sample sizes. The aim of this study was to investigate sex‑based differences in the clinical phenotype at diagnosis of SjD and identify predictors of patient sex using a large international cohort and AI‑enhanced analysis. METHODS:Cross-sectional analysis of an anonymised dataset comprising 17,416 worldwide patients fulfilling the 2002/2016 classification criteria (Sjögren Big Data Registry). We stratified the dataset by sex and conducted a comparative analysis of baseline glandular and systemic involvement, organ-specific ESSDAI domains, and immunological profiles. Multivariate logistic regression models were developed, adjusting for epidemiological confounders (age and ethnicity) to identify predictors of sex classification. We used a generative AI (OpenAI's GPT-4o model) environment with Python (version 3.9) and the pandas (1.4.3), numpy (1.21.5), and matplotlib (3.5.1) libraries. All analyses adhered to GDPR standards, with anonymized patient data and strictly controlled secure environments. RESULTS:The cohort included 1,161 (6.67%) men and 16,255 (93.33%) women, with a mean age at diagnosis of 51.11 years (SD=14.45). Men showed a higher mean age at diagnosis (54.09 vs. 51.42 years in women; t=6.08, p<0.0001), a higher average ESSDAI score (7.65 vs. 5.93; t=7.91, p<0.0001) and higher frequencies in severe DAS categories (i.e. high activity 20% vs. 12% in women, χ² = 81.15, p<0.0001). The epidemiologically-adjusted logistic regression model (pseudo R-squared value of 0.026) identified statistical significance for age (coefficient =0.009, p=0.024; each additional year in age increased the likelihood of being female by 1.4%), ethnicity (coefficient=0.579, HR=1.78, p=0.004), ocular dryness (coefficient=-0.607, HR=0.54, p<0.001), and systemic activity in the glandular (coefficient=0.359, HR=1.43, p=0.006) and pulmonary (coefficient=0.445, HR=1.56, p=0.004) ESSDAI domains. CONCLUSIONS:Male SjD patients present a distinct, more systemic phenotype at diagnosis. Awareness of sex‑specific features can improve early recognition and tailored management.

OBJECTIVES:This study aimed to analyse the relationship between distinct autoantibody combinations (immunological signatures) and systemic disease activity in patients with Sjögren's disease (SjD). The hypothesis was that specific multi-autoantibody signatures would be associated with higher systemic disease activity at diagnosis, serving as predictors of a more severe disease course. METHODS:A retrospective observational study was conducted using data from the Big Data Sjögren Project Consortium, an international multicentre registry. The serological status (positive/negative) at diagnosis for ANA, RF, anti-Ro, and anti-La was recorded for each patient. Systemic disease activity was assessed using the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and a simplified Disease Activity Score (DAS) categorised as low, moderate, or high. Statistical analyses included pairwise comparisons, a sensitivity analysis grouping signatures by the number of positive antibodies, and demographic-adjusted ordinal models. RESULTS:Serum autoantibodies were highly prevalent, with over 94% of patients having at least one autoantibody. The mean ESSDAI values varied significantly across signatures. The fully seronegative group had the lowest mean ESSDAI at 3.61, while the fully seropositive group (ANA+/Ro+/La+/RF+) had the highest among common phenotypes, with a mean of 7.93. A strong dose-response relationship was observed, with each additional positive autoantibody associated with a 1.11-point mean increase in ESSDAI and a 35% increase in the odds of being in a higher DAS category. The rarest signatures, such as ANA-/Ro-/La+/RF+, exhibited the highest mean systemic activity (mean 13.20). CONCLUSIONS:The number and combination of SjD-related autoantibodies at diagnosis are robustly associated with systemic disease activity. Multi-positive profiles, particularly those combining RF with anti-Ro, identify patients at higher risk of systemic activity. Interpreting combined serological patterns offers an immediate, low-cost method for patient stratification and can help guide clinical management.

Sjögren's disease (SjD) is a chronic systemic autoimmune disorder that primarily involves lymphocytic infiltration of exocrine glands, with frequent extra-glandular manifestations. Historically, treatment options for SjD have been limited to alleviating symptoms, rather than treating the underlying cause or preventing disease progression. Furthermore, past clinical trials of therapies such as rituximab failed to demonstrate improvement in symptoms or disease activity. Recently, novel therapeutic strategies targeting underlying disease pathogenesis - including transcription factors, circulating RNA, and B and T cell activity - herald a paradigm shift. Given the complexities of diagnosis, clinical assessment and treatment in SjD, improved clinical trial design with enhanced patient stratification, greater inclusivity and better outcome measures are paramount in evaluating new therapeutics. This systematic review aims to provide a comprehensive overview of recent SjD therapeutic advances, assess trial inclusivity with respect to sex/gender and ethnicity, critically examine negative pivotal trials and highlight promising directions for future research.

Fine mapping and bioinformatic analysis of the DDX6-CXCR5 genetic risk association in Sjögren's Disease (SjD) and Systemic Lupus Erythematosus (SLE) identified five common SNPs with functional evidence in immune cell types: rs4938573, rs57494551, rs4938572, rs4936443, rs7117261. Functional interrogation of nuclear protein binding affinity, enhancer/promoter regulatory activity, and chromatin-chromatin interactions in immune, salivary gland epithelial, and kidney epithelial cells revealed cell type-specific allelic effects for all five SNPs that expanded regulation beyond effects on DDX6 and CXCR5 expression. Mapping the local chromatin regulatory network revealed several additional genes of interest, including lnc-PHLDB1-1. Collectively, functional characterization implicated the risk alleles of these SNPs as modulators of promoter and/or enhancer activities that regulate cell type-specific expression of DDX6, CXCR5, and lnc-PHLDB1-1, among others. Further, these findings emphasize the importance of exploring the functional significance of SNPs in the context of complex chromatin architecture in disease-relevant cell types and tissues.

Background: Sjögren's disease (SjD) is a complex systemic autoimmune disease with substantial morbidity and 21 known genetic associations. The International Sjögren's Genetics Network (SGENE) is a growing international collaboration focused on understanding how genetic variants influence SjD pathology. As sample sizes increase, we are focusing our efforts on the analyses of clinical subsets, which few studies have done. Objectives: Our genome-wide association study (GWAS) aimed to identify additional risk loci of genome-wide significance (GWS, p<5E-08; suggestive, p<5x10E-5) in European-derived subsets of SjD. Methods: This study was conducted with IRB/EC approvals. All SjD patients met the 2002 AECG criteria for SjD. A total of 5058 cases and 25943 controls were genotyped on GWAS arrays. After QC, 4855 cases and 25408 controls were included in the analyses. Logistic regression was calculated, adjusting for ancestry using the first 4 principal components to identify SjD-associated SNPs. Cases were split into Ro/SSA+ (n=2898) and Ro/SSA- (n=1313), and analyzed vs. each other, controls, and all-SjD. Results: We observed many differences in the genomic architecture of Ro/SSA- compared to Ro/SSA+ and all SjD (Figure 1a,b), most notably, a complete loss of the significance of the association with MHC on chromosome 6 in the Ro/SSA- cases(Figure 1b). The Ro/SSA+ subjects had a much stronger HLA association with OR ≈ 4 (Figure 1a), while the overall SjD showed OR ≈ 3. While none of the associations observed in the Ro/SSA- population reached GWS, 8 regions (near PLXNA2, PCDH7, IRF5-TNPO3, DLD, LOC100134229, JAK3, LOC643529, and TMTC1) show suggestive associations (Figure. 1a). Of these, only two, IRF5-TNPO3 and LOC643529, are also suggestive in the Ro/SSA+ subset. However, while the Ro/SSA+ have both the IRF5 promoter effect and the extended haplotype through TNPO3, the Ro/SSA- lack the IRF5 promoter effect. Interestingly, previous studies have shown that lupus and systemic sclerosis have both haplotypes while primary biliary cholangitis only has the haplotype extending into TNPO3, similar to Ro/SSA- SjD [1]. When comparing Ro/SSA- to the all-SjD dataset, PLXNA2 and LOC100134229 showed no association; PCDH7, DLD, and TMTC1 showed some association but did not reach suggestive levels; and LOC643529, IRF5-TNPO3, and JAK3 surpassed the suggestive threshold, the latter two nearing or surpassing GWS. Two of the novel suggestive associations in Ro/SSA- are particularly intriguing. PLXNA2 is a member of a semaphorin co-receptor family that mediates repulsive effects on axon pathfinding during nervous system development; interestingly, Ro/SSA- SjD has a higher frequency of neurological involvement. JAK3 is a member of the Janus kinase (JAK) family of tyrosine kinases involved in cytokine receptor-mediated intracellular signal transduction; it is predominantly expressed in immune cells. Mutations in this gene are associated with autosomal severe combined immunodeficiency disease. Novel drugs target the JAK-STAT pathways, making this finding markedly relevant. Conclusion: Our findings highlight the relevance of expanding genetic studies to specific subphenotypes of the disease. While we continue to increase our GWAS sample size and explore other subphenotypes, more work is needed to increase the power of these studies to determine if the suggestive regions will surpass the GWS threshold. REFERENCES: [1] Kottyan LC, et al. Hum Mol Genet. 2015 Jan 15;24(2):582-96. Acknowledgements: NIH/NIAMS R01 AR073855, P50 AR060804; NIH/NIDCR U01DE028891; Sjögren's Foundation; Jerome L. Greene Foundation. Disclosure of Interests: Astrid Rasmussen: None declared, Marcin Radziszewski: None declared, Bhuwan Khatri: None declared, Kandice L Tessneer: None declared, Elena Pontarini: None declared, Michele Bombardieri: None declared, Maureen Rischmueller: None declared, Marie Wahren-Herlenius: None declared, Marika Kvarnström: None declared, Torsten Witte: None declared, Hendrika Bootsma: None declared, Gwenny M. Verstappen: None declared, Frans G.M. Kroese: None declared, Arjan Vissink: None declared, Sarah Pringle: None declared, Athanasios Tzioufas: None declared, Clio Mavragani: None declared, Alan Baer Received consulting fees from Bristol Myers Squibb (BMS) and iCell Gene Therapeutics., Marta Alarcon-Riquelme: None declared, Javier Martin: None declared, Xavier Mariette: None declared, Gaetane Nocturne: None declared, Jacques-Olivier Pers: None declared, Jacques-Eric Gottenberg: None declared, Wan-Fai Ng I have consulted for Novartis, BMS, Janssen, Sanofi, Abbvie, IQVIA, Argenx, Resolve Therapeutics., Caroline Shiboski: None declared, Kimberly E Taylor: None declared, Lindsey Criswell: None declared, Blake M Warner: None declared, A Darise Farris Grant/research support from Johnson and Johnson Innovative Medicine (formerly Janssen; ended 12/31/2023)., Judith A. James: None declared, R Hal Scofield Received consulting fees from Johnson and Johnson Innovative Medicine (formerly Janssen) and Merk Pharmaceuticals., Joel M Guthridge: None declared, Daniel J Wallace: None declared, Swamy Venuturupali: None declared, Michael T Brennan: None declared, Juliana Imgenberg-Kreuz: None declared, Lars Ronnblom: None declared, Eva Baecklund: None declared, Maija-leena Eloranta: None declared, Lara A Aqrawi: None declared, Øyvind Palm: None declared, Johan G Brun: None declared, Daniel Hammenfors: None declared, Malin V Jonsson: None declared, Silke Appel: None declared, Sara Magnusson Bucher: None declared, Helena Forsblad-d'Elia: None declared, Thomas Mandl Employee of UCB., Per Eriksson: None declared, Gunnel Nordmark: None declared, Christopher J Lessard Grant/research support from Johnson and Johnson Innovative Medicine (formerly Janssen; ended 12/31/2023).

Background: Sjögren disease (SjD) is a chronic autoimmune disorder characterized by inflammation of the exocrine glands, particularly the salivary and lacrimal glands, leading to dryness of the eyes and mouth. Current therapeutic options for SjD are empirical, due to the lack of evidence-based recommendations. However, off-label use of biologics or small molecules, particularly B-cell targeted therapies, is applied in some clinical scenarios in SjD. Research on the use of targeted therapies in SjD is ongoing, and several clinical trials are exploring their efficacy. Objectives: To analyse the off-label use of biologic and small molecule therapies in treating complicated/severe SjD using real-world data. Methods: The Big Data Project Consortium is an international, multicentre registry created in 2014. The inclusion criteria were the fulfilment of the 2002/2016 classification criteria. By December 2023, the participant centers had included 16703 valid patients from 28 countries. We have retrospectively analysed the use of biological and small molecule therapies in SjD, including the pharmacological classification of the used drugs, therapeutic indications, sequential and combination use, and causes of mortality. Results: The use of biologics and small molecules was reported in 330 (2%) patients, who received 359 therapeutic courses. We identified 23 different therapies that overwhelmingly consisted of B-cell targeted drugs (83%); more rarely, the drugs used targeted T cells (8%), tyrosine kinases (2.5%), TNF (2.5%), cytokines (2%), integrins (0.6%), and complement (0.3%). B-cell targeted therapies overwhelmingly consisted of rituximab (79%), followed by belimumab (4%), and isolated cases of use of obinutuzumab and ofatumumab (<1%). T-cell targeted therapies included the use abatacept (8%); a large list of other biologics and small molecule inhibitors (16 different drugs) were used in less than 5 cases per drug. Combined biologic therapies were reported in only four patients (belimumab plus rituximab). The main therapeutic indication consisted of ESSDAI severe involvements that predominantly affected a single organ (57%), followed by the treatment of lymphoma (23%), and by severe systemic multiorgan involvement (18%). According to the predominantly affected ESSDAI domain, besides lymphadenopathy, the most frequent indications included articular (20%), pulmonary (7%), PNS (6%), CNS (6%), and cutaneous (5%) involvements (Figure 1). Death was reported in 32 patients (10%), with the most frequent causes being infection (34%) and cancer progression (31%). It should be noted that 4 (36%) of the 11 patients who died from infection were caused by COVID-19 (all of them were under treatment with rituximab). Conclusion: The real-life use of biologic and small molecule therapies in patients with SjD is exceptional (2%), a fact closely related to their off-label use in most therapeutic indications. Nearly 85% of therapies are directed against B cells (overwhelmingly rituximab), with exceptional cases of new anti-CD20 monoclonal antibodies or combined therapy with rituximab and belimumab. Mortality is higher than expected in SjD treated with the biologic and small molecule therapies [1], infections (notably COVID-19 in patients treated with rituximab) and cancer progression being the most frequent causes of death, thus highlighting the need for defining advanced treatment indications in SjD. REFERENCES: [1] Brito-Zeron P, et al. EClinicalMedicine. 2023; 61(4):102062. doi: 10.1016/j.eclinm.2023.102062 Acknowledgements: MD Juliana Viana Baiao Lemos, collaborator from Rheumatology Department, Hospital das Clinicas da Faculdade de Medicina de Ribeirão Preto Universidade de Sao Paulo, Brazil. Disclosure of Interests: None declared.Figure 1

Autoimmune rheumatic disease (AIRD) is a collective term, which comprises a group of multisystem inflammatory autoimmune diseases, including connective tissue disease, chronic inflammatory arthritis, sarcoidosis and systemic vasculitis. Some AIRD are prevalent in the general population, and all can cause significant morbidity and reduced quality of life, with some increasing the risk of premature mortality, such as systemic lupus erythematosus (SLE), a connective tissue disease that is more prevalent and severe in Australian Aboriginal and Torres Strait Islander Peoples with high mortality rates. To ensure that management of AIRD can be optimised for all Australians, it is important that we understand the prevalence and potential phenotypic variations of AIRD across the Australian population. However, to date there have been few described cases of AIRD other than SLE in Aboriginal and Torres Strait Islander Peoples. In this review, we summarise what is known about AIRD other than SLE in Aboriginal and Torres Strait Islander Peoples, particularly with regards to prevalence, phenotype and disease outcomes, and highlight the current gaps in knowledge.