Gastrointestinal involvement in very early and established systemic sclerosis: insights from the SPRING-SIR national Italian registry.

Objectives: To describe the prevalence of gastrointestinal (GI) symptoms in systemic sclerosis (SSc) and Very Early Diagnosis of SSc (VEDOSS), identify clinical and serological features associated with GI involvement, and explore a cranio-caudal pattern of symptom distribution, using data from the Italian SPRING-SIR registry.

Methods: This cross-sectional analysis included patients fulfilling 2013 ACR/EULAR SSc or VEDOSS criteria. GI involvement was defined as symptoms in at least one GI tract segment and categorized as upper and lower.

Deep hierarchical subtyping of multi-organ systemic sclerosis trajectories - a EUSTAR study.

Systemic sclerosis (SSc) is a chronic autoimmune disease with multi-organ involvement. Historically, SSc classification has focused on the type of skin involvement (limited versus diffuse); however, a growing evidence of organ-specific variability suggests the presence of more than two distinct subtypes. We propose a semi-supervised generative deep learning framework leveraging expert-driven definitions of organ-specific involvement and severity.

Aquaporin-4 downregulation in dysphagic patients with idiopathic inflammatory myopathies: myofiber vulnerability and inflammation drivers.

Objectives: To evaluate whether the impairment of Aquaporin-4 (AQP4), a water channel protein important for muscle function, may be associated with dysphagia in idiopathic inflammatory myopathies (IIMs). Swallowing muscle inflammation in IIM has been documented in case reports, but direct correlations with limb muscles impairment are lacking.

Methods: AQP4 was immunolocalized in limb muscle biopsies.

Beyond inflammation: the molecular basis of bone remodeling in axial spondyloarthritis and psoriatic arthritis.

Spondyloarthritis (SpA) encompasses a group of chronic inflammatory diseases with overlapping genetic, clinical, and radiographic features. Axial spondyloarthritis (axSpA), a subset of SpA, predominantly involves the sacroiliac joints and spine, often progressing to ankylosis, severe disability, and functional impairment. Psoriatic arthritis (PsA), another SpA subtype, is characterized by a heterogeneous phenotype that includes peripheral arthritis, enthesitis, and axial involvement, frequently associated with psoriasis.

Targeting axial and peripheral psoriatic arthritis: a retrospective observational study on the clinical relevance of upadacitinib.

Objective: To evaluate upadacitinib (UPA) effectiveness on axial and peripheral manifestations of psoriatic arthritis (PsA) by assessing the proportion of patients achieving low disease activity (LDA) and inactive disease (ID) status for axial involvement, and MDA and DAPSA-defined remission/LDA for peripheral domain.

Methods: This retrospective study included PsA patients from 27 Italian rheumatology centers. Demographic, clinical, and outcome data were collected at baseline, 6, and 12 months.

Managing the clinical heterogeneity of patients with Still's disease, from early diagnosis to timely treatment.

Still's disease is an inflammatory syndrome affecting patients across all ages, previously known as systemic juvenile idiopathic arthritis (sJIA) in children and adult-onset Still's disease (AOSD) in adults. Multiple lines of evidence reported overlapping clinical features between sJIA and AOSD, commonly manifesting with daily fever, arthritis, evanescent salmon-coloured skin rash. The concomitant various degree of multiorgan involvement may increase the heterogeneity of the patient clinical picture.

Menopause in systemic sclerosis: the impact on clinical presentation in a multicenter cross-sectional analysis from the National Registry of the Italian Society for Rheumatology (SPRING-SIR).

Background: Hormonal changes in menopause might interact with the presentation of underlying autoimmune diseases, such as systemic sclerosis (SSc).

Objectives: Our study aimed to evaluate the association of (1) current menopausal status, (2) early menopause, and (3) disease onset during fertile or post-menopausal age on SSc clinical phenotype in a large SSc cohort from the Italian Systemic sclerosis Progression INvestiGation (SPRING-SIR) registry.

Design: Female SSc patients from the SPRING-SIR registry, fulfilling the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 classification criteria, with data on SSc disease onset, menopausal status, and menopausal age, were eligible.

Serum ferritin as a specific biomarker of anti-MDA5-interstitial lung disease: a multicenter, case-control study in observational and validation datasets.

Background: Serum ferritin has been proposed as biomarker of interstitial lung disease (ILD) in anti-MDA5 dermatomyositis (DM). Nevertheless, no data exist for serum ferritin in other idiopathic inflammatory myopathies (IIM)-ILD nor in IIM without ILD. Aim of this case-control study was to assess whether serum ferritin may be considered a specific and sensitive biomarker for IIM-ILD, as well as to assess whether it correlates with clinical and immunological findings.

First-line diagnostic tests to intercept primary heart involvement in systemic sclerosis: Clinical associations from the SPRING-SIR registry.

Introduction: Primary heart involvement (pHI) is an overlooked and poorly characterised complication of systemic sclerosis (SSc), associated with the risk of heart failure, arrhythmia and death. Despite consensus definition by the World Scleroderma Foundation/Heart Failure Association (WSF/HFA), diagnostic criteria and risk factors remain poorly elucidated.

Methods: Out of 1922 patients in the Italian national SPRING registry, we excluded those with potentially confounding conditions according to WSF/HFA, and those with incomplete ECG or echocardiographic assessment, resulting in 600 subjects with clearly defined parameters to intercept SSc-pHI.

Incidence and prevalence of systemic lupus erythematosus across Italian referral centers and its clinical burden in terms of disease severity, treatment and hospitalization: The ESCULAPIO study.

Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan involvement. Limited data from specialized referral centers are available at national level. This study assessed epidemiologic, clinical, therapeutic features and hospitalization burden of SLE through data from tertiary Italian referral centers.

Italian Multicenter Real-World Study on the Twelve-Month Effectiveness, Safety, and Retention Rate of Guselkumab in Psoriatic Arthritis Patients.

: Psoriatic arthritis (PsA) is a chronic inflammatory condition that primarily affects the musculoskeletal system and skin. While biologic and targeted synthetic DMARDs have improved treatment, many patients still fail to achieve remission. Combining conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) with biologic (b) DMARDs or targeted synthetic (ts) DMARDs shows no added benefit over monotherapy with IL-17, IL-23 inhibitors, or JAK inhibitors, unlike TNFi, which benefit from csDMARD co-administration.

Lung involvement in psoriatic arthritis patients: cross-sectional study on prevalence, clinical characteristics, and risk factors of a neglected comorbidity.

Objectives: We aimed to assess lung involvement in patients with psoriatic arthritis (PsA), hypothesising that lung diseases (LDs) represent a distinct comorbidity of PsA and that the association between these two conditions is not merely coincidental.

Methods: Cross-sectional study of 322 patients with a diagnosis of PsA from three Italian centres. Each patient underwent a chest high-resolution CT prior to the start of a biological DMARDs.

Upadacitinib for the treatment of radiographic axial spondyloarthritis - case series and review of the literature.

Upadacitinib is a Janus kinase (JAK) inhibitor approved for the treatment of different rheumatic diseases, including axial spondyloarthritis (axSpA). In phase III clinical trials, upadacitinib was associated with rapid and significant improvement in disease parameters, including scores for pain, function and mobility, signs of structural damage, and patient-reported outcomes, and had an overall incidence of adverse events similar to that in the placebo group. Improvement in axSpA disease severity was observed in both biologic-naive patients and those with prior biologic exposure, and this improvement was sustained during open-label treatment.

Prevalence and clinical relevance of digital ulcers in systemic sclerosis patients from the real-life: the experience of the SPRING Registry of the Italian Society for Rheumatology.

Introduction: Digital ulcers (DU) are one of the most frequent manifestations in systemic sclerosis (SSc). The presence of DU seems to be a sentinel sign of internal organ involvement and is related to a poor prognosis of the disease. The aim of this study was to evaluate the prevalence and the relationship of DU with clinical manifestations/variants in a large SSc cohort from the SPRING registry.

Kinesiophobia as part of the psychological burden in Spondyloarthritis: a case-control study.

Objectives: Psychological distress is commonly reported by patients affected by Spondyloarthritis (SpA), with >50% experiencing concomitant depression or anxiety. This case-control study aimed to investigate the psychological dimensions of SpA by assessing and comparing levels of kinesiophobia, depression and health-related quality of life (HRQoL) between SpA patients and a healthy control (HC) group.

Methods: This cross-sectional case-control study included patients with SpA classified by Assessment of SpondyloArthritis international Society classification criteria and a group of HCs matched by sex and age.

Disease burden in inflammatory arthritis: an unsupervised machine learning approach of the COVAD-2 e-survey dataset.

Objectives: To comprehensively compare the disease burden among patients with RA, PsA and AS using Patient-Reported Outcome Measurement Information System (PROMIS) scores and to identify distinct patient clusters based on comorbidity profiles and PROMIS outcomes.

Methods: Data from the global COVID-19 Vaccination in Autoimmune Diseases (COVAD) 2 e-survey were analysed. Patients with RA, PsA or AS undergoing treatment with DMARDs were included.

Single-camera motion capture of finger joint mobility as a digital biomarker for disease activity in rheumatoid arthritis.

Objective: To investigate the association between hand motion tracking features obtained through computer vision from smartphone cameras and disease activity in patients with RA.

Methods: The PyPI package of MediaPipe (version 0.9.

Disparities in the organisation of national healthcare systems for treatment of patients with psoriatic arthritis and axial spondyloarthritis across Europe.

Background: Studies on national policies for biologics are warranted.

Objectives: To map and compare national healthcare set-ups for prescription, start, switch, tapering, and discontinuation of biologic/targeted synthetic disease-modifying antirheumatic drugs (DMARDs) in patients with psoriatic arthritis and axial spondyloarthritis across Europe, and assess the healthcare set-ups in relation to countries' socio-economic status.

Methods: An electronic survey was developed to collect and compare information on national healthcare systems.

Incidence of Major Adverse Cardiovascular Events in Patients With Rheumatoid Arthritis Treated With JAK Inhibitors Compared With Biologic Disease-Modifying Antirheumatic Drugs: Data From an International Collaboration of Registries.

Objective: Our objective was to assess the incidence of major adverse cardiovascular events (MACEs) in patients with rheumatoid arthritis (RA) treated with JAK inhibitors (JAKi), tumor necrosis factor inhibitors (TNFi), or biologic disease-modifying antirheumatic drugs with other modes of action (bDMARD-OMA) in a multicountry, real-world population.

Methods: Patients with RA from 15 registries in the JAK-pot collaboration were included. MACE incidence was analyzed using two approaches: a within-registry analysis aggregating country-specific estimates from registers with >25 incident MACEs through meta-analysis and an individual-level data combined analysis.

Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies.

Objective: Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASS). Treatment typically involves high-dose corticosteroids (CCS) and conventional synthetic disease-modifying antirheumatic drugs (csDMARD). Rituximab (RTX) has shown effectiveness in refractory cases.

Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study.

Background: Following the results of the MANDARA trial, this real-life study aimed at comparing the effectiveness and safety profile of mepolizumab versus benralizumab in a European EGPA cohort.

Methods: We conducted a retrospective observational comparative study including EGPA patients, who received mepolizumab or benralizumab at the asthma dose. Patients were matched 1:1 by sex, age, BVAS and oral corticosteroid (OCS) dosage at the treatment initiation (T0).

Efficacy and safety of conventional disease-modifying antirheumatic drugs in VEXAS syndrome: real-world data from the international AIDA network.

Background: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory condition resulting in severe, often treatment-refractory inflammation. Currently, there are no established treatment guidelines for VEXAS syndrome.

Objectives: To assess the efficacy and safety of conventional disease-modifying antirheumatic drugs (cDMARDs) in a cohort of VEXAS patients.

Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry.

Background: VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context.