BACKGROUND:Anifrolumab is a type I interferon receptor antagonist approved for the treatment of systemic lupus erythematosus (SLE). However, real-world evidence on its use, especially from large, unselected cohorts, is scarce. The ongoing REVEAL study is designed to collect real-world data on anifrolumab use. The data reported here are the pre-specified 6-month interim analysis, which aims to provide a phenotypic characterisation of a large real-world cohort of patients with SLE initiating anifrolumab, and to evaluate early treatment response in routine clinical practice. METHODS:REVEAL is a 5-year, multicentre, prospective observational study conducted in 25 tertiary rheumatology centres across Italy. A pre-specified interim analysis was planned when the first 50 patients completed the first 6 months of follow-up; this analysis includes all patients who initiated anifrolumab by the data cutoff of Feb 10, 2025. Patients with SLE were consecutively enrolled on the day of their first infusion of anifrolumab, prescribed according to clinical judgement and Italian indications for use. Eligible patients were aged 18 years or older, had a clinical diagnosis of SLE fulfilling at least one set of established classification criteria valid at the time of diagnosis (1997 American College of Rheumatology [ACR], 2012 Systemic Lupus International Collaborating Clinics, or 2019 European Alliance of Associations for Rheumatology-ACR), had active disease warranting anifrolumab treatment (including compassionate use programmes), and were naive to anifrolumab. Data were collected at baseline and at 1 month, 3 months, and 6 months. The primary outcome was the number of patients reaching remission (defined according to the Definition of Remission in SLE criteria as a clinical SLEDAI-2K score of 0, physician global assessment score of <0·5 [on a 0-3 scale], with a prednisone-equivalent dose ≤5 mg per day, and stable antimalarials or immunosuppressants), Lupus Low Disease Activity State (LLDAS; defined as a SLEDAI-2K ≤4 [with no activity in major organ systems and no new disease activity], physician global assessment ≤1·0, and a prednisone-equivalent dose ≤7·5 mg per day), and LLDAS5 (a modified version of the LLDAS with a prednisone-equivalent dose ≤5 mg per day) at 6 months. Adverse and serious adverse events were also recorded. No people with lived experience of SLE were involved in designing or conducting the study. This study is registered with ClinicalTrials.gov (NCT07215754) and with the Italian Medicines Agency (Agenzia Italiana del Farmaco; ID number 247) and recruitment is ongoing. FINDINGS:Between May 25, 2023, and Feb 10, 2025, 236 patients were recruited and included in this interim analysis. Of these, 219 (93%) were female, 17 (7%) were male, 218 (92%) were White, and the median age was 46·9 years (IQR 36·0-53·6). At baseline, the median SLEDAI-2K was 7 (IQR 6-9), and the main indications for anifrolumab were mucocutaneous (157 [67%]) and articular (116 [49%]) involvement. At 6 months, 37 (26%) of 140 patients reached remission, 80 (57%) reached LLDAS5 and 93 (66%) reached LLDAS. One patient was excluded from the outcome analysis due to missing physician global assessment data. 108 adverse events were recorded during the 6-month follow-up period; of these, 83 (77%) were infections. Five serious adverse events occurred, resulting in six hospitalisations. INTERPRETATION:This study provides the first large-scale real-world evidence of anifrolumab use in patients with SLE, supporting its clinical benefit and rapid onset of action in routine care. FUNDING:None.

OBJECTIVES:To assess the utility of lung magnetic resonance imaging (MRI) for monitoring interstitial lung disease (ILD) in patients with systemic sclerosis (SSc), using high-resolution CT (HRCT) as the reference. Additionally, we explored associations between MRI sequences and common imaging and functional parameters in SSc-ILD evaluation. METHODS:SSc patients with ILD requiring treatment initiation or change underwent lung assessment at baseline and after 6 months, including MRI (T2-weighted, T1 post-contrast, and T2 star sequences), HRCT, lung ultrasound, and pulmonary function tests. Six-month MRI and HRCT were qualitatively evaluated for improvement, stability, or progression. A follow-up HRCT was performed 2 years after enrolment. RESULTS:Fourteen SSc-ILD patients (64.3% female, mean age 48.3 years) were enrolled. MRI showed improvement in 1 patient, stability in 9, and progression in 4; in 2 progressed cases, inflammation decreased while fibrotic features increased. T1 contrast sequence significantly correlated with pleural irregularities on ultrasound (ρ=0.55; p=0.04) and DLCO (ρ=-0.65; p=0.01). MRI and HRCT findings at 6 months were concordant in 64.3% of cases, with fair agreement (weighted κ=0.25). MRI outcomes at 6 months matched HRCT findings at 2 years in 92.8% of patients (13/14). CONCLUSIONS:Lung MRI is a promising adjunctive tool for monitoring SSc-ILD and may provide complementary information to HRCT on early imaging changes, particularly the transition from inflammation to fibrosis. Among MRI sequences, T1 post-contrast best correlated with functional and ultrasound findings, supporting its role in fibrosis assessment.

OBJECTIVES:Sarcopenia, defined by the European Working Group on Sarcopenia in Older People 2 (EWGSOP2) as loss of muscle strength, mass and function, is an underestimated complication in systemic sclerosis (SSc). We aimed to assess sarcopenia prevalence in SSc, identify clinical correlations, and evaluate its prognostic impact. METHODS:Consecutive adult SSc patients underwent multidisciplinary same-day assessment (rheumatologists, physiotherapists, nutritionists) and HAQ administration. Sarcopenia was diagnosed using the EWGSOP2 algorithm: SARC-F questionnaire and/or clinical suspicion for screening, muscle strength testing for probable sarcopenia, confirmation with reduced appendicular skeletal muscle mass (by bioelectrical impedance vector analysis [BIVA]), and severity by impaired physical performance. Malnutrition was assessed by BIVA. Mortality was recorded after ≥12 months follow-up. RESULTS:Among 204 enrolled patients (86.8% female, mean age 62.3 years), 100 (49%) were at risk of sarcopenia. Of these, 89 (43.6%) had reduced muscle strength (probable sarcopenia), 54 (26.5%) showed low muscle mass (confirmed sarcopenia), and 25 (12.3%) had impaired physical performance (severe cases). Sarcopenic patients were older (p<0.001) and had higher HAQ scores (p<0.001). Malnutrition was identified in 69/204 patients (33.8%) and was more prevalent in sarcopenic patients (72.2% vs. 24.6%, p<0.001). Sarcopenia was strongly associated with malnutrition (OR 5.69, p<0.001). Mortality was significantly higher in sarcopenic patients (16.7% vs. 2.7%, p=0.001), who also showed reduced survival (p<0.001). Sarcopenia predicted mortality (HR 8.99, p=0.001), remaining an independent predictor after adjustment for age (HR 4.09, p=0.04). CONCLUSIONS:Sarcopenia is frequent in SSc, closely associated with malnutrition, disability and mortality. Sarcopenia is an independent prognostic factor, therefore routine screening and multidisciplinary management are warranted.

OBJECTIVES:Understanding the molecular events underlying systemic lupus erythematosus (SLE) onset and progression can facilitate early intervention strategies. We explored transcriptomic changes during progression from preclinical to clinical and advanced SLE, and assessed their potential reversibility by targeted agents. METHODS:This includes multicentre prospective study of individuals with nondiagnostic features suggestive of SLE. Baseline blood RNA-sequencing was performed in groups who progressed to classifiable SLE or not (n = 36 each), and compared with healthy (n = 42) and early SLE (n = 43). Transcriptome analysis included supervised methods, gene module-based clustering, and drug reversibility/repurposing pipelines with publicly available data. An independent at-risk cohort (n = 15 progressors, n = 20 nonprogressors) was used to validate molecular classifiers. RESULTS:At-risk individuals exhibited deregulated metabolic, cytokine signalling, haematologic, and stress-response pathways. Progressors vs nonprogressors showed heightened interferon (IFN)-alpha (α)/gamma (γ) and inflammatory cytokine activity, corroborated by Gene Set Enrichment Analysis and coexpression analysis, and intensified during SLE classification. Unsupervised modelling of gene module-eigengene patterns revealed molecular heterogeneity among progressors, differing in p53/insulin activity and Toll-like receptor (TLR) signalling. Importantly, we characterised a 17-gene susceptibility signature that predicted transition with an area under the curve 0.80 of the receiver operating characteris (95% CI: 0.65-0.94) in the external cohort. Analysis across the continuum-from healthy and at-risk states to early SLE and lupus nephritis-revealed stepwise upregulation of IFN-α/γ, haem metabolism, and oxidative phosphorylation pathways, with additional IFN-related genes activated in established disease. SLE susceptibility and progression signatures demonstrated in silico reversibility by anifrolumab and belimumab. CONCLUSIONS:IFN-α/γ and inflammatory cytokine signatures characterise evolving/early SLE, whereas amplification of IFN signalling and oxidative phosphorylation denotes severity. Blood molecular profiling may aid risk stratification and rationalise early biologic approaches.

OBJECTIVES:Lymphoid interstitial pneumonia (LIP) is a rare form of Interstitial Lung Disease (ILD), often associated with Sjögren Disease (SjD). However, the clinical-serologic characteristics of SjD-LIP remain poorly characterized. Our objective was to describe the clinical course and outcome of SjD-associated LIP and to compare this subgroup with other SjD-ILD patterns. METHODS:SjD patients with High-resolution Computed Tomography (HRCT)-confirmed ILD followed in Pisa Rheumatology Unit (January 2019-November 2024) were retrospectively enrolled. ILD patterns were classified through multidisciplinary discussion. Clinical and laboratory data were collected according to ESSDAI definitions, along with pulmonary symptoms and function tests (PFTs). RESULTS:Fifty-five SjD-ILD patients were included (M: F = 9:46), of whom 11 were diagnosed with LIP (F: M = 11:0). LIP patients showed thin-walled parenchymal cysts as the predominant HRCT finding, and largely preserved pulmonary function (median forced vital capacity [FVC] 101% [IQR 98-105]; DLCO 76% [IQR 75-81]). After a median 5-years follow-up (IQR 2-7) all LIP patients were alive with stable PFTs. Compared with the remaining 44 non-LIP, LIP patients were younger at SjD diagnosis (P < 0.001) and more frequently presented purpura (P = 0.012), constitutional symptoms (P = 0.001), lymphadenopathy (P = 0.023), hypergammaglobulinemia (P < 0.001), triple anti-Ro60/52/La positivity (P = 0.035) and C3 hypocomplementemia (P = 0.009). ILD preceded SjD diagnosis in 30/44 non-LIP vs. 1/11 LIP patients (P < 0.001), with lower FVC% (P = 0.049) and DLCO% (P = 0.036) in non-LIP. CONCLUSION:LIP defines a distinct, immunologically active phenotype within the spectrum of SjD-ILD, characterized by greater extrapulmonary systemic involvement and serologic markers of B cell hyperactivity, but limited pulmonary functional impact. These findings support long-term lymphoma surveillance and a potential role for B cell targeted therapies in selected patients.

Patients with rare diseases are particularly vulnerable during emergencies due to dependence on specialised care pathways, medicines, and expertise. European Reference Networks (ERNs) for rare and complex diseases have operated since 2017 and were confronted with two major crises: the COVID-19 pandemic and the war in Ukraine. To assess ERN experiences, responses, and preparedness for crisis and disaster situations, we conducted a cross-sectional survey of all 24 ERN coordinators between July and September 2025. Only 2 (8·3%) ERNs reported pre-existing preparedness plans, while 70% lack structured frameworks. Major bottlenecks included patient access to healthcare facilities, drug and device shortages, bed shortage, staff unavailability, and diagnostic service interruption. Our findings highlight the need to formally integrate and mandate ERNs into European health emergency preparedness and response mechanisms. We propose a 10-point global crisis preparedness plan for rare diseases emphasizing cross-border coordination, digital infrastructure, patient education, and integration with national emergency services and non-governmental organizations.

Introduction Chimeric antigen receptor (CAR) T-cell therapy has emerged as a transformative therapeutic option for patients with severe, refractory autoimmune conditions. Since 2022, several authors have published their experience using CAR T-cell therapy for patients with systemic lupus erythematosus (SLE). Methods This narrative review summarizes the biological rationale, diverse modalities, targets and platforms, the emerging clinical efficacy and safety data, as well as the barriers and innovations regarding the use of CAR T-cell therapy in SLE. Results Among the 16 studies reviewed, 13 evaluated products targeting CD19 and the remaining three bispecific products targeting CD19 and B-cell maturation antigen (BCMA). Twelve studies assessed autologous therapies and four allogeneic products. Therapies evaluating CAR natural killer cells (CAR-NK), gamma delta T-cells (γδ T-cells), other transduction methods, and even in vivo CARs are currently being evaluated. Early data has demonstrated significant clinical efficacy, including rapid disease activity reductions, drug-free remission in over 80%, achievement of low-level disease activity in almost 90%, and remission in 70% of the patients where these outcomes were assessed. Cytokine release syndrome occurred in 56% of the patients evaluated, almost exclusively being grade 1 or 2 events, and neurotoxicity was rare. Conclusions CAR T-cell therapy has demonstrated encouraging clinical efficacy and reassuring safety outcomes so far. However, longer prospective studies are needed to assess durability of response, long term safety, factors associated with poor response, and the appropriate selection and timing of patients for therapy.

OBJECTIVES:In a cross-sectional study, we aimed at characterizing possible impairments in sleep health and rest-activity parameters between patients with Sjögren's Disease (SjD) and healthy controls (HCs). Furthermore, we explored possible predictors of such disturbances in the SjD group. METHODS:Participants' sleep and rest-activity rhythms were assessed via 7-day continuous accelerometry, the Pittsburgh Sleep Quality Index, the Epworth Sleepiness Scale, and the reduced Morningness-Eveningness Questionnaire, allowing the creation of a multidimensional sleep health index. Parametric tests explored between-group differences in sleep and rest-activity parameters, while functional linear modelling characterized between-group, time-related differences in accelerometric activity. Within the SjD cohort, regression analysis was employed to explore disease activity, patient-reported disease burden, and the Hospital Anxiety and Depression Scale (HADS) as possible predictors of sleep and rest-activity parameters. RESULTS:Forty-six SjD patients and forty age-, sex- and BMI-matched HCs were included. Compared with HCs, SjD patients reported lower sleep health (P = 0.005) and delayed mid-sleep point (P = 0.009) and acrophase (P = 0.033). Functional linear modelling confirmed the objective shift towards eveningness in SjD patients. In SjD patients, patient-reported disease burden predicted sleep quality (β = 0.41; P = 0.044) and sleepiness (β = 0.83; P = 0.010), while disease activity predicted daily steps (β=-526; P = 0.013), total sleep time (β = 0.15; P = 0.0496) and sleep regularity (β=-1.3; P = 0.030). Finally, HADS predicted daily steps (β = -238; P = 0.041), total sleep time (β = -0.08; P = 0.035) and sleep efficiency (β = -0.65; P = 0.012). CONCLUSIONS:SjD is associated with impaired sleep health and rest-activity rhythms. In SjD patients, such alterations differentially associate with disease activity and patient-reported outcomes, supporting a multifactorial model of sleep and circadian rhythms disruption.

Systemic lupus erythematosus (SLE) guidelines predominantly focus on common major organ involvement. An international taskforce from three SLE expert groups (European Reference Network on Connective Tissue and Musculoskeletal Diseases, Systemic Lupus International Collaborating Clinics, and the European Lupus Society) previously developed consensus therapeutic strategies for 24 rare SLE manifestations. Here, 77 participants contributed to the development of consensus therapeutic strategies for 22 additional rare SLE manifestations, including diffuse pulmonary haemorrhage, rare cutaneous manifestations (bullous lupus, chilblain lupus, lupus tumidus, erythema multiforme, and toxic epidermal necrolysis-like lupus erythematosus), renal manifestations (interstitial nephritis and lupus podocytopathy), rare neurological manifestations (chorea, small fibre neuropathy, catatonia, and intracranial hypertension), rare gastrointestinal manifestations (protein-losing enteropathy, lupus hepatitis, intestinal pseudo-obstruction, and peritonitis), musculoskeletal manifestations (myositis and Jaccoud's arthropathy), and other rare manifestations such as uveitis, angioedema due to anti-C1 esterase inhibitor antibodies, interstitial cystitis, and lupus mastitis. These expert-based therapeutic strategies provide a framework for guiding therapeutic decisions where evidence-based recommendations might be insufficient.

The ReCONNET-ELICIT methodology represents an innovative and systematic approach to gather expert consensus on complex, high-level concepts related to rare connective tissue and musculoskeletal diseases (rCTDs). Developed within the framework of European Reference Networks (ERNs) ReCONNET, this methodology aims to overcome the challenges associated with defining complex features that lack consensus in rCTDs, such as composite definitions for severe or refractory disease. The core premise of RECONNET-ELICIT is that individual expertise, including that of experts, is inherently limited, but that aggregating insights from a sufficiently large and diverse group can achieve extensive and relevant facet elicitation. The RECONNET-ELICIT process begins with assembling a diverse panel from various geographical backgrounds. This inclusive approach ensures that multiple perspectives are considered. The next step involves crowdsourcing responses through an online survey, where participants suggest as many facets of the high-level concept as possible. These proposals are then systematically analyzed and recoded into broader categories through a consensus-driven binning process. Following this, the panel reviews the consolidated list of facets and votes on their final inclusion, typically utilizing rating scales to minimize bias. This approach is particularly valuable in rCTD contexts where limited data are available. Overall, ReCONNET-ELICIT aim at enhancing the clarity and shared understanding of complex disease concepts by integrating expert insights within an asynchronous, structured, participatory framework while avoiding dominance bias. Its flexibility and focus on collective agreement make it especially useful for advancing knowledge and improving management strategies for rare CTDs. What is already known on this topic Rare connective tissue and musculoskeletal diseases (rCTDs) often lack standardized criteria and consensus definitions, especially for complex high-levels concepts such as severe, refractory disease or rare manifestations. What this study adds The ReCONNET-ELICIT methodology offers an innovative, systematic approach to gather expert participation and consensus on complex, high-level concepts in rCTDs, where robust evidence is typically lacking, leveraging diverse and comprehensive perspectives, from an expert panel. How this study might affect research, practice, or policy By systematically capturing facets of high-level complex concepts through expert consensus, the ReCONNET-ELICIT methodology can improve clinical guidelines, guide future research efforts and inform policy development, ultimately enhancing rCTD patient care.

Abstract Background Although individual rare and complex diseases (RDs) affect small patient populations, together they impact an estimated 27–36 million people across the European Union. Addressing this major public health challenge has been a long-term priority for the European Union, leading to the establishment of the European Reference Networks (ERNs) in 2017. Main body ERNs are cross-border networks connecting clinical expert centres to share knowledge, improve and harmonise diagnosis and care for patients with rare and complex diseases. Since their inception, 24 ERNs have united 1,606 expert centres across 375 hospitals in all EU Member States and Norway. Their activities span multidisciplinary clinical collaboration, patient-centred governance, education and training, and the development of clinical guidelines. Over 4900 extremely rare or difficult cases have been discussed among experts without requiring the patients to travel abroad when expertise was not available in their own countries. A key factor for this success is the cross-border IT platform - known as the Clinical Patient Management System 2.0 - provided by the European Commission for medical discussions, which enables experts to share patient data, including medical images and lab results, in a secure and protected environment that is fully compliant with all relevant security and data privacy requirements. ERNs have demonstrated resilience in crises such as the COVID-19 pandemic and the war in Ukraine, providing rapid, coordinated responses to sustain care for vulnerable patient groups. The first formal evaluation in 2023 confirmed that more than 95% of member centres met quality standards, underscoring the networks’ maturity and effectiveness. Moving into the next phase, the Joint Action JARDIN (2024–2027) aims to integrate ERNs into national healthcare systems to ensure sustainability and equitable access to high-quality RD care. Conclusions ERNs exemplify European solidarity and innovation in healthcare, transforming how rare disease expertise is shared and applied across borders. Their continued integration into national systems will be pivotal to achieving a truly cohesive European Health Union that delivers improved outcomes for all patients with rare and complex diseases.

BackgroundWhile Sjögren disease (SjD) overlap is known to modulate the clinical-serologic manifestations of other connective tissue diseases, the association with anti-synthetase syndrome (ASyS) has been seldom described in Caucasian cohorts. Anti-Ro52 autoantibodies, shared by both conditions, may blur early diagnostic attribution, particularly when glandular symptoms precede myositis-spectrum features.ObjectivesTo characterize the clinical phenotype, serological profile, and disease trajectory of patients fulfilling classification criteria for both SjD and ASyS.MethodsWe conducted a multicentre retrospective study (2018-2025) across three Italian referral centres, including patients meeting both the 2016 ACR/EULAR SjD criteria and the Connor’s/provisional CLASS classification criteria for ASyS. Clinical features, serology, interstitial lung disease (ILD) characteristics, treatments, and outcomes were systematically collected. SjD activity was assessed using the ESSDAI and an adapted ESSDAI excluding the classic ASyS triad (pulmonary, articular and muscular domains).ResultsSeventeen female patients of Caucasian ethnicity were identified. At the time of connective tissue disease diagnosis, 7 were classified as SjD and 10 received concomitant diagnoses; all reported early sicca symptoms. Regarding serology, anti-Ro52 antibodies were detected in all patients, most often (88%) in the absence of anti-Ro60 antibodies. Anti-synthetase antibodies were present in all cases, mainly non-Jo-1 specificities (77%), while anti-Jo-1 was observed in a minority of patients (23%). ILD was the leading organ involvement (14/17, 82%), typically with acute/subacute onset and NSIP or NSIP/OP patterns. ILD drove treatment decisions and accounted for four deaths, whereas most survivors showed stabilization or mild improvement under immunosuppressive treatment. Systemic assessment indicated a predominantly ASyS-driven phenotype: although ESSDAI was moderately elevated (median 15, IQR 7.5-21), the adapted ESSDAI markedly decreased (median 5, IQR 0-6.5), reflecting limited SjD-specific systemic activity.ConclusionsThe SjD-ASyS overlap seem characterized by a coherent clinical-serological phenotype, defined by anti-Ro52-positive sicca presentation, non-Jo1 anti-ARS autoantibodies and early interstitial lung disease. Pulmonary involvement represents the main driver of disease course and outcomes in this subset. Recognition of this pattern may improve early identification of ASyS in Ro52-positive SjD presentations. Further prospective studies are warranted to clarify whether this subset reflects a distinct phenotype within the ASyS spectrum rather than a coincidental overlap of two independent diseases.

Background: Inflammatory bowel diseases (IBD), including Crohn’s disease (CD) and ulcerative colitis (UC), are chronic conditions that affect the gastrointestinal tract. Since the initial approval of infliximab (IFX), a monoclonal antibody targeting TNF-α, numerous novel therapeutic targets have been identified, and many new therapies have been approved for the treatment of IBD. Methods: We conducted a narrative review of the literature using major biomedical databases, including EMBASE, Scopus, PubMed, CENTRAL, and ClinicalTrials.gov (last search date: 10 December 2025). Results: This review summarizes the current evidence on therapies approved for IBD (both CD and UC) and provides an overview of investigational agents currently being evaluated in ongoing phase II and III clinical trials. Conclusions: Moderate optimism arises from the expanding array of therapeutic targets under investigation and from emerging treatment strategies. However, only through a deeper understanding of the pathophysiological mechanisms underlying IBD will substantial improvements in treatment outcomes be achieved for conditions that continue to impose a significant burden on patients’ quality of life.

Objectives This study aimed to develop a set of quality indicators (QIs) based on the 2025 European Alliance of Associations for Rheumatology (EULAR) recommendations for systemic lupus erythematosus with kidney involvement and explore their association with disease outcomes. Methods A modified Research and Development and University of California, Los Angeles appropriateness method was used. In 2 voting rounds, 23 experts rated candidate QIs for validity and feasibility. Median ratings and agreement were calculated, leading to a core QI set. Adherence to recommended care from healthcare providers was assessed at both QI and patient levels in a single tertiary centre contemporary lupus nephritis (LN) cohort (n = 130). High adherence was defined as ≥80%. Associations between adherence to QIs, damage accrual, and renal flares were explored. Results A total of 17 QIs were developed across 4 domains: diagnosis, treatment, monitoring, and pregnancy. High adherence was observed for kidney biopsy, hydroxychloroquine use, maintenance immunosuppression, therapy switching in persistent or relapsing disease, renin–angiotensin system inhibitor use, remission before kidney transplantation, and pregnancy-related QIs. Combination immunosuppressive therapy as initial treatment had low adherence (10.7%), reflecting practice patterns preceding publication of the recommendations. High per-patient adherence was associated with lower odds of damage accrual (increase in Systemic Lupus International Collaborating Clinic Damage Index; odds ratio: 0.29, 95% CI: 0.13-0.62) and fewer renal flares, although the latter association was not statistically significant. Conclusions These QIs may serve as a structured framework for implementing the 2025 EULAR recommendations for LN. Higher adherence was associated with reduced damage accrual in a tertiary centre cohort, but results need validation in diverse clinical settings.

Little is known about the relationship between thyroid diseases (TDs) and Idiopathic inflammatory myopathies (IIMs). This study aimed at evaluating the prevalence of TDs in a monocentric cohort of patients with IIMs, exploring possible correlations with clinical phenotype, organ involvement, comorbidities and quality of life (QoL). We retrospectively analysed medical records of patients with IIM according to the EULAR/ACR 2017 criteria, collecting data about demography, IIM subset, disease duration, autoantibody profile, organ involvement and comorbidities. Besides, we registered the occurrence of Hashimoto Thyroiditis (HT), Multinodular goitre (MNG), Grave’s Disease (GD) and Thyroid papillary cancer (TPC). In addition, some different patient reported outcomes were administered, to collect data on QoL. A total of 191 patients were enrolled: 125 (65.4