Nailfold videocapillaroscopy findings and associations with organ involvement in mixed connective tissue disease.

Objectives: To investigate nailfold videocapillaroscopy (NVC) abnormalities in mixed connective tissue disease (MCTD).

Methods: Patients with MCTD followed at the Rheumatology Department in Cochin Hospital (Paris, France) were identified based on individual record review. Diagnosis of MCTD required fulfillment of one of the three sets of classification criteria.

Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis.

Background And Aims: Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments.

Performance of the EULAR Systemic sclerosis Impact of Disease (ScleroID) questionnaire as a patient-reported outcome measure for patients with diffuse systemic sclerosis.

Objective: Systemic sclerosis Impact of Disease (ScleroID) is the first comprehensive patient-reported outcome measure (PROM) specifically developed for systemic sclerosis (SSc). We investigated the performance of ScleroID in patients with diffuse cutaneous SSc (dcSSc), as a prerequisite for its use in randomised controlled trials (RCTs) testing potentially disease-modifying drugs.

Methods: All patients with dcSSc from the large, multicentric, ScleroID cohort were included.

Results from the international collaborative systematic literature review informing the 2023 EULAR recommendations for the treatment of systemic sclerosis.

Background: The EULAR recommendations for the treatment of systemic sclerosis (SSc) were updated in 2017, informed by a systematic literature review (SLR) completed in 2014.

Objectives: The aim of this new SLR was to provide the most up-to-date literature to underpin contemporary EULAR recommendations for the management of SSc.

Methods: 30 searches for 30 interventions (including several outcomes/clinical questions), and 1 dedicated search (with several interventions) for calcinosis were prioritised by the task force.

Real-world assessment of the efficacy and tolerability profile of JAK inhibitors in difficult-to-treat rheumatoid arthritis.

Objective: To evaluate the effectiveness and tolerability of JAK inhibitors (JAKi) in patients with difficult-to-treat rheumatoid arthritis (D2TRA) in clinical practice.

Methods: We included RA patients initiating a JAKi between 2018 and 2022. Patients meeting EULAR criteria for D2TRA were compared to active non-D2TRA patients.

Association of serum interferon alpha-2a levels with disease severity and prognosis in systemic sclerosis.

Objective: To determine serum type I interferon IFN-α2a concentrations in systemic sclerosis (SSc) patients, explore its association with cytokine/chemokine expressions, and evaluate correlation with the phenotype including the predictive value for interstitial lung disease (ILD) progression.

Methods: Serum samples were obtained from 200 SSc patients and 29 healthy controls. IFN-α2a levels were measured by ultrasensitive electrochemiluminescence assay.

A randomised, parallel-group, double-blind, placebo-controlled phase 3 study to Determine the effectiveness of the type I interferon receptor antibody, Anifrolumab, In SYstemic sclerosis: DAISY study design and rationale.

Objectives: The type I interferon pathway is a promising target for treatment of patients with systemic sclerosis (SSc). Here, we describe the design of a multinational, randomised phase 3 study to Determine the effectiveness of the type I interferon receptor antibody, Anifrolumab, In SYstemic sclerosis (DAISY).

Methods: DAISY includes a 52-week double-blind, placebo-controlled treatment period, a 52-week open-label active treatment period, and a 12-week safety follow-up period.

Tailoring the treatment of inflammatory rheumatic diseases by a better stratification and characterization of the clinical patient heterogeneity. Findings from a systematic literature review and experts' consensus.

Inflammatory rheumatic diseases are different pathologic conditions associated with a deregulated immune response, codified along a spectrum of disorders, with autoinflammatory and autoimmune diseases as two-end phenotypes of this continuum. Despite pathogenic differences, inflammatory rheumatic diseases are commonly managed with a limited number of immunosuppressive drugs, sometimes with partial evidence or transferring physicians' knowledge in different patients. In addition, several randomized clinical trials, enrolling these patients, did not meet the primary pre-established outcomes and these findings could be linked to the underlying molecular diversities along the spectrum of inflammatory rheumatic disorders.

Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists.

Objectives: The objectives were to explore rheumatologists' current clinical screening practices of pulmonary arterial hypertension in patients with systemic sclerosis in the United Kingdom and to identify barriers to screening and consider potential solutions.

Methods: A survey of 31 questions was developed and included six sections: clinician demographics, the importance of screening, screening practices, barriers to screening, treatment and patient education. The survey was disseminated among rheumatologists working in the United Kingdom.

Evaluation of the Rheumatoid Arthritis Impact of Disease (RAID) Score in Assessing Rheumatoid Arthritis Activity in Teleconsultation.

Objective: To evaluate the relevance of the Rheumatoid Arthritis Impact of Disease (RAID) score as a disease activity marker of rheumatoid arthritis (RA) in a teleconsultation setting.

Methods: A prospective, observational, 24-month, single-center study involving patients with RA who underwent teleconsultations was performed. The RAID score was sent to all patients by email and completed the day before the scheduled session.

Biomarker analysis from the phase 2b randomized placebo-controlled trial of riociguat in early diffuse cutaneous systemic sclerosis.

Objective: To examine disease and target engagement biomarkers in the RISE-SSc trial of riociguat in early diffuse cutaneous systemic sclerosis and their potential to predict the response to treatment.

Methods: Patients were randomized to riociguat (n = 60) or placebo (n = 61) for 52 weeks. Skin biopsies and plasma/serum samples were obtained at baseline and week 14.

The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis-Interstitial Lung Disease in the European Scleroderma Trial and Research Database.

Background And Objectives: In SSc, ILD is a major cause of morbidity and mortality. We aimed to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) delta change (Δ) and baseline values in predicting the development of SSc-ILD.

Methods: Longitudinal data of DLCO, FVC, and ILD on the HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t) and after 12 (±4) (t) and 24 (±4) (t) months.

Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis.

Objective: Systemic sclerosis is an autoimmune disease characterized by fibrosis of the skin and internal organs including the lung. Mucosal-associated invariant T cells are innate-like T lymphocytes able to produce various cytokines and cytotoxic mediators such as granzyme B. A large body of evidence supports a role of mucosal-associated invariant T cells in autoimmune disease but more recent reports suggest also a potential role in fibrotic conditions.

The role of skin ultrasound in systemic sclerosis: looking below the surface to understand disease evolution.

Skin ultrasound has shown promising results in the evaluation of skin involvement in patients with systemic sclerosis, as substantiated by a recent systematic literature review from the World Scleroderma Foundation Skin Ultrasound Working Group. In this Viewpoint, we will discuss the role of ultrasound in evaluating skin involvement in patients with systemic sclerosis, particularly the possibility of using this technique to detect an early subclinical skin involvement from the very early phase, suggesting its possible use in both diagnosis and disease follow-up. To detect subclinical skin involvement, it is essential to understand the difference between the skin of patients with systemic sclerosis and that of healthy controls, including defining exactly which structures are affected by the disease and which are spared.

Digital Ulcers and Ventricular Arrhythmias as Red Flags to Predict Replacement Myocardial Fibrosis in Systemic Sclerosis.

Background: Cardiac involvement in systemic sclerosis (SSc) affects the prognosis of the disease. Echocardiography is the first line imaging tool to detect cardiac involvement, but it is not able to routinely detect myocardial fibrosis. Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is the gold standard for replacement myocardial fibrosis assessment, but its availability is currently limited.