Objectives Digital ulcers (DUs) affect ∼50% of systemic sclerosis (SSc) patients, causing significant pain and functional impairment. Current management involves both systemic and local therapies. However, the burden in terms of pain and quality of life due to refractory DUs still remains heavy. While selexipag is approved for SSc-associated pulmonary arterial hypertension, its potential in treating

Objectives Safety is a concern for solid-organ transplant (SOT) recipients with systemic inflammatory diseases (SID) treated with biologic therapy. This study evaluated the safety of biologic therapy in SOT recipients with SID. Methods This retrospective study between 2000–2024 included 20 biologic-treated SOT recipients with SID matched to 56 SOT recipients controls without SID not treated with biologic

Objectives Patients with anti-threonyl (PL7) positive anti-synthetase syndrome (ASS) exhibit a high prevalence of interstitial lung disease (ILD), which can progress to progressive pulmonary fibrosis (PPF) and increased mortality. This study aims to explore the clinical characteristics, imaging features, and predictive factors for PPF. Methods A retrospective cohort of PL7-ASS patients at a single

Objectives Osteoarthritis (OA) presents a significant clinical challenge due to its heterogeneous nature, characterized by cartilage degradation, inflammation, and fibrosis. Current treatments offer limited efficacy, highlighting the need for novel therapeutic approaches. Our study aimed to investigate the effects of two JAK inhibitors, tofacitinib and baricitinib, on various hallmarks of OA in human

Objectives Epithelial-Stromal Interaction 1 (EPSTI1), an interferon-related gene that has emerged as a gene of notable interest, plays a multifaceted role in cellular function and disease processes. However, the precise role of EPSTI1 in the context of dermatomyositis(DM) remains elusive and requires further exploration. Methods To investigate EPSTI1 expression in DM, we analyzed two transcriptome

Objective Interstitial pneumonia with autoimmune features (IPAF) describes patients with interstitial lung disease (ILD) and autoimmune features without meeting criteria for a specific rheumatic disease. No longitudinal data exist on post-transplant outcomes in IPAF patients. We compared baseline demographics, pre-transplant characteristics, and post-transplant outcomes between IPAF and idiopathic

Objectives Cardiovascular disease is a leading cause of mortality in systemic sclerosis (SSc). We investigated the association between SSc and the occurrence of both cardiovascular and thromboembolic events using the Clinical Practice Research Datalink (CPRD). Methods A validated case-ascertainment strategy identified SSc patients in the CPRD. A cohort study design examined rates of coronary arterial

Objectives To investigate the ex vivo IFN-γ release assay (IGRA) as a biomarker of SLE activity and disease outcome. Methods This retrospective study, conducted between 2008 and 2024 at a single tertiary care center, included 145 SLE patients at various disease stages. Data were collected on spontaneous IFN-γ levels (IGRA-nil) and on phytohemagglutinin-induced IFN-γ levels (IGRA-PHA, after subtracting

Objectives Our aim was to explore the effect of a single subcutaneous dose of CAM2043, a novel extended-release subcutaneous formulation of treprostinil, on finger temperature in patients with systemic sclerosis (SSc)-related Raynaud’s phenomenon (RP). Methods This was an exploratory, open-label, single-dose Phase 2 trial. Ten female patients (median age 54.0 years) attended on 6 occasions: screening

Objective To identify risk factors for spinal structural progression and determine the most significant impact on syndesmophytes growth and impaired spinal mobility in axSpA. Methods Baseline and 2-year follow-up clinical, thoracic and lumbar spine CT data of 94 patients were analyzed. Vertebral syndesmophytes unit (VSU) was defined as syndesmophytes anterior to vertebral body and its intervertebral

Objective Idiopathic inflammatory myopathies (IIM) are autoimmune disorders characterised by muscle inflammation, high creatine kinase (CK) levels, and disability. Despite immunomodulating therapies, patients often experience progressive disease, resulting in refractory conditions or dependence on glucocorticoids (GC). This study addresses the efficacy and safety of immunoadsorption (IAS) in refractory

Objectives Given a substantial proportion of patients with ASS-ILD (anti-synthetase syndrome-associated interstitial lung disease) develop progressive pulmonary fibrosis (PPF), we aimed to investigate the clinical-radiographic characteristics and treatment in patients with or without anti-Jo-1 antibody. Methods Patients diagnosed with ASS-ILD from two centers were retrospectively reviewed and stratified

Objective The impact of primary Sjögren’s syndrome (pSS) on adverse pregnancy outcomes remains a debated issue. Research suggests that newborns of mothers with pSS may be at a higher risk of developing heart conditions. This study aimed to examine the relationship between maternal pSS and the risk of cardiac disorders in neonates. Methods A multicentre, retrospective cohort study was conducted with

Objectives Patients with rheumatoid arthritis (RA) are at increased risk of atherosclerotic cardiovascular disease (ASCVD), yet traditional 10-year CVD risk calculators—such as the 2013 Pooled Cohort Equations (PCE)—underperform in RA. Our objective was to determine the performance of the PCE vs the updated PREVENT algorithm in indicating presence of subclinical coronary and carotid atherosclerosis

Objective This study aimed to assess the efficacy and safety of baricitinib in Takayasu arteritis (TAK) refractory to TNF-α inhibitors. Methods We conducted a multicentre, single-arm trial between February 2021 and August 2023. Patients with TAK unresponsive to at least 6 months of TNF-α inhibitors were treated with baricitinib 4 mg daily for up to 48 weeks, while continuing of immunosuppressants and

Objectives To evaluate the efficacy and safety of Janus kinase inhibitors (JAKi) in a monocentric series of patients with JDM and to identify factors associated with the achievement of clinically inactive disease (CID). Methods Single-centre retrospective study of 39 JDM patients treated with JAKi for at least 6 months. The proportion of patients achieving CID within 6 months after initiation of JAKi

Objectives To determine the effect of paternal and maternal anti-IL-1 treatment exposures on pregnancies and neonatal outcomes in CAPS. Methods A single-centre study consecutive adult CAPS patients and their partners was performed between January 2012 and July 2024. All were screened for pregnancies and anti-IL-1 exposure before conception and/or during pregnancy; teratogenic co-therapies resulted

Objective Antinuclear antibody (ANA) positivity has been observed in patients with steatotic liver disease (SLD); however, the link between SLD and ANA-positive connective tissue diseases remains unexplored. We aimed to evaluate the association between SLD and risk of systemic sclerosis (SSc), a representative ANA-positive connective tissue disease. Methods A longitudinal population-based cohort study

Objectives The gut microbiota is crucial in the progression of lupus nephritis (LN), and probiotics have emerged as a potential therapeutic approach. This study investigates the role of Bifidobacterium in LN pathogenesis and its effects on immune cell populations. Methods Clinical samples were collected from 61 systemic lupus erythematosus (SLE) patients, and flow cytometry was used to analyze changes

Objectives Recent European League Against Rheumatism guidelines highlight the importance of shared decision-making (SDM) in systemic lupus erythematosus (SLE) treatment. This study investigated the relationship between physicians' personality traits and patient-rated SDM. Methods Using baseline data from the Trust Measurements for Physicians and Patients with SLE (TRUM2-SLE) study, physicians' personality

Objectives Current recommendations suggest treating eosinophilic granulomatosis with polyangiitis (EGPA) without severe manifestations with glucocorticoids (GCs) and EGPA with severe manifestations with GCs plus cyclophosphamide (CYC) regardless of poor-prognostic factors. However, GCs plus CYC and GCs alone have never been compared in EGPA without poor-prognosis factors assessed by the 1996 Five Factor

Objectives To characterize some phenotypic and functional aspects of fibroblast-like synoviocytes isolated from the synovial fluid (SF-FLS) of patients affected by Juvenile Idiopathic Arthritis (JIA) with active disease and to compare SF-FLS characteristics to those reported in the literature for FLS of the synovial membrane (SM) in adult rheumatic patients. Methods FLS were isolated from the SF of

Objectives Interstitial lung disease (ILD) is the most frequent cause of mortality in patients with systemic sclerosis (SSc). Cartilage acidic protein-1 (CRTAC1), secreted by alveolar type-2 epithelial cells, is a potential biomarker of lung health. This study explores CRTAC1's role in SSc, focusing on pulmonary involvement. Methods We collected plasma samples from 76 SSc patients (65 females, limited

Objectives Evidence for the association between methotrexate use and liver fibrosis in psoriatic arthritis (PsA) remains inconclusive. We aimed to explore the frequency of liver fibrosis in PsA and identify associated factors including cumulative methotrexate dose and metabolic features. Methods We analyzed data from a prospective observational PsA cohort. We calculated the Aspartate Aminotransferase

Objectives Mitochondria are prominent antigenic sources, capable of triggering interferon-induced inflammatory pathways in systemic lupus erythematosus (SLE). Recent studies suggest presence of anti-mitochondria autoantibodies (AMA) in lupus nephritis patients with adult-onset SLE. Whether AMA reflect disease activity in childhood-onset SLE (cSLE) remains unexplored. Here, we present inaugural data

Objectives Body composition plays a significant role in various rheumatic and autoinflammatory diseases. The aim of our study was to assess the impact of muscle mass and subcutaneous adipose tissue quality and quantity in patients with systemic sclerosis (SSc). Methods Adults with SSc referring to our tertiary center who underwent high-resolution chest computed tomography (HRCT) to assess pulmonary

Objectives To characterize the incidence of clinically diagnosed Paget’s disease of bone (PDB) in Castille and Leon, a region in the central plateau of Spain, from 2009 to 2019, and explores its association with environmental factors, particularly livestock activity. Methods We analyzed primary care electronic medical records to assess PDB incidence trends over time and geographic differences across

Objectives To evaluate change in body weight with disease-modifying antirheumatic drugs (DMARD) in psoriatic arthritis (PsA). Methods We analyzed data from a large cohort of PsA patients with at least two weight measurements over follow-up. The absolute weight difference at one year from drug initiation was evaluated across—no medications or non-steroidal anti-inflammatory drugs (NSAIDs), conventional

Objectives The role of neutrophil extracellular traps (NETs) in immunoglobulin A vasculitis (IgAV) pathogenesis is emerging, with NETosis-associated markers potentially linked to disease activity. This study aimed to explore the relationship between NETosis biomarkers and IgAV disease phases. Methods A longitudinal study involving 33 pediatric IgAV patients and 26 healthy controls was conducted. Blood

Objectives ANCA-associated vasculitis (AAV) is a group of systemic autoimmune diseases affecting small blood-vessels. Class-II HLA genes often reported as major genetic determinants. We conducted a systematic review and meta-analysis to evaluate the susceptibility conferred by HLA genes in AAV and five sub-types i.e. PR3+AAV, MPO+AAV, Granulomatosis with polyangiitis (GPA), Microscopic polyangiitis

Objectives The Fibrosis-4 index (FIB-4), a non-invasive tool for assessing liver fibrosis, has also been linked to cardiovascular (CV) risk in the general population. This connection is due to the association of chronic liver diseases, particularly fibrosis or non-alcoholic fatty liver disease, with systemic inflammation, metabolic syndrome, and atherosclerosis. In this study, we aimed to calculate

Objectives Cognitive dysfunction is a common neuropsychiatric manifestation in systemic lupus erythematosus (SLE), particularly affecting processing speed and memory. This study aims to identify behaviorally relevant topological networks of functional connectivity underlying neuropsychological test performances, using connectome-based predictive modeling (CPM). Methods 43 SLE patients and 34 controls

Objectives We systematically reviewed the literature to identify the incidence of psoriatic arthritis (PsA) flare, criteria used to define it, and associated risk factors. Methods Databases of Embase, Medline ALL, Web of Science Core Collection and Cochrane Central Register of Controlled Trials were searched until September 2023, for original articles studying PsA flare. The Newcastle Ottawa scale

Objective Application of the ASAS classification criteria for axSpA in classifying axPsA is a topic of debate. In this study, we aimed to determine the prevalence of axPsA in patients with psoriasis and back pain who do not meet the entry pain features of the ASAS classification criteria. Methods Patients reporting late-onset back pain (LoBP, after the age of 45) or non-chronic back pain (NcBP, lasting

Objectives To evaluate the discordance between patients’ global assessments (PGA) and evaluators’ global assessments (EGA) in psoriatic arthritis (PsA), and to explore the association of clinical variables, patient-reported outcomes (PROs), and ultrasound (US) factors with this discordance. Methods Consecutive PsA patients (CASPAR criteria) were prospectively recruited and evaluated by a comprehensive

Objectives For IFN-driven diseases, such as juvenile dermatomyositis (JDM), there is a critical need for targeted therapies. We aimed to develop an in vitro model, using Siglec-1 as read-out, to evaluate inhibition of IFN-mediated responses with different JAK inhibitors (JAKi). Methods Healthy donor (HD) PBMCs were cultured with type I and II IFNs, TLR agonists, and plasma or serum from patients (JDM

Objectives This study aims to analyze the expression profiles, phenotypes, functions, and cell–cell communication of various cell subpopulations in the affected aortic tissues of patients with Behçet's syndrome (BS) at the transcriptomic level. Methods This study recruited six participants (three with Behçet's syndrome and three with atherosclerosis) from Beijing Anzhen Hospital between January 2023

Objectives B cell depletion therapy has been used for over two decades to treat systemic lupus erythematosus (SLE), but there is a lack of studies reporting its impact on damage progression. This study aims to assess the effectiveness of rituximab in slowing damage acquisition. Methods We selected 380 patients 190 treated with rituximab and 190 controls, based on matched sex and age of onset, with

Objectives To evaluate the effectiveness and safety of secukinumab in patients with giant cell arteritis (GCA) who experienced an inadequate response to tocilizumab. Methods This is a case series of six patients with GCA started on secukinumab after tocilizumab failure from three centres in Italy. Tocilizumab failure was defined as disease relapse, indicated by clinical symptoms and/or vascular inflammation

Objective To elucidate the prevailing concerns by analyzing the shifts in clinical profiles and therapeutic outcomes of patients with rheumatoid arthritis from 2003 to 2022, and offer guidelines to refine future treatment approaches. Methods Utilizing our registry data, we conducted an analysis of the patient background and biennial variations in the clinical remission rate one year post-initiation

Objectives Recent guidelines and recommendations for lupus nephritis (LN) suggest rapid glucocorticoid (GC) reduction; however, robust supporting evidence remains limited. This study aimed to evaluate the impact of rapid GC reduction on renal outcomes in patients with proliferative LN. Methods We conducted a multicentre retrospective chart review of patients with GC-naïve, biopsy-proven proliferative

Objectives We aimed to assess the safety and efficacy of ultrasound-guided needle muscle biopsy (UGNMB) using a 14G biopsy needle in obtaining adequate samples for histological analysis and establishing an inflammatory idiopathic myopathy (IIM) diagnosis in patients with suspected myositis. Methods We included patients who were followed up at our Rheumatology Department with a suspected IIM diagnosis

Objectives To analyse clinical features and biomarkers associated with primary heart involvement (pHI) confirmed at cardiac magnetic resonance (CMR) in a monocentric cohort of systemic sclerosis (SSc) patients. Methods A retrospective study. SSc-patients who underwent CMR for clinically suspected pHI were identified. SSc-pHI at CMR was defined by the presence of at least one abnormality among: non-ischemic

Objectives Immune-mediated necrotising myopathy (IMNM) with autoantibodies targeting 3-hydroxy-3-methylglutaryl-CoA reductase (anti-HMGCR) is considered a rare complication of statin therapy. We calculate the incidence of anti-HMGCR IMNM and describe clinical characteristics in four independent cohorts: Manchester (UK), Bristol (UK), Western Australia (WA, Australia) and South Australia (SA, Australia)

OBJECTIVE To investigate the prevalence, timing, and risk factors at diagnosis associated with relapses in giant cell arteritis (GCA), with a separate analysis of major and minor relapses. METHODS A retrospective review of patients from the Spanish multicentre GCA registry (ARTESER) was conducted. Relapses were classified using 2018 EULAR definitions. RESULTS We included 1,284 patients with at least