Objective: We (1) compared anxiety symptom levels in a multinational systemic sclerosis (SSc) cohort to a general population normative sample and (2) evaluated sociodemographic, lifestyle, and SSc disease factors associated with symptoms.
Methods: Scleroderma Patient-centered Intervention Network Cohort participants completed the Patient-Reported Outcomes Measurement Information System (PROMIS) Version 2 4a Anxiety domain upon enrolment. PROMIS domain scores use T-scores (mean = 50, standard deviation = 10) calibrated to a United States normative sample.
Objective: To compile a list of approved training sites for the Residency Training Program [Diplôme d'Études Spécialisées] in Internal Medicine and Clinical Immunology (DES-MIIC) in France.
Method: All local coordinators of the DES-MIIC were contacted to establish the list of approved internship sites for the MIIC DES within their geographical subdivision.
Results: We listed 244 approved training sites, of which 87 (35.
Objective: To determine distinct patterns of patients with autoimmune diseases harbouring anti-Ku antibodies and their respective prognosis.
Methods: Anti-Ku-positive patients were retrieved through four immunology departments. Clusters were derived from unsupervised multiple correspondence analysis, not including the disease's diagnosis, followed by hierarchical clustering.
Objectives: Systemic sclerosis (SSc) is characterised by widespread vascular damage resulting in digital and systemic vasculopathic sequelae. Although there are effective treatments available, vascular disease remains a significant cause of morbidity and mortality in SSc. Our aim was to describe patterns of vascular medication use in SSc, including examination for potential changes over time.
View Article and Find Full Text PDFRheumatology (Oxford)
May 2025
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 Score (FFS). We aimed to compare the efficacy of GCs plus CYC vs GCs alone for the treatment of EGPA without poor-prognosis, including among patients with severe manifestations.
View Article and Find Full Text PDFBackground: Research results are often not communicated to study participants or others with relevant lived experience. Effective communication of research results would help study participants understand their contribution to research and could improve trust in research and likelihood of research participation. Few randomized controlled trials (RCTs), however, have compared the effectiveness of research communication tools, and it is not known which tools work best for different people.
View Article and Find Full Text PDFRev Med Interne
May 2025
Introduction: The prevalence of systemic sclerosis (SSc), as well as obesity, has significantly increased in recent decades. To address the lack of data on obese SSc patients, we conducted a retrospective comparative study to assess the prevalence, clinical characteristics, and long-term consequences of obesity in SSc patients.
Methods: We conducted a retrospective comparative study at the Cochin University Hospital's Department of Internal Medicine (Paris) from 2000 to 2019.
Introduction: Mixed connective tissue disease (MCTD) is a rare systemic disorder that belongs to connective tissue diseases (CTD). Few studies are available on MCTD treatment.
Methods: We conducted an observational study within the French MCTD cohort.
Introduction: Scleroderma renal crisis (SRC) is a major vascular complication of systemic sclerosis (SSc), associated with high morbidity and mortality. In this retrospective study, we evaluated the potential prognostic and diagnostic roles of angiogenesis molecules, placental growth factor (PlGF), soluble fms-like tyrosine kinase 1 (sFlt-1) and sFlt1/PlGF ratio as biomarkers in SRC.
Methods: Sera samples from 27 patients with a history of SRC (SSc-SRC+) were collected following event occurence.
J Scleroderma Relat Disord
March 2025
Background: Interstitial lung disease is the leading cause of morbidity and mortality in systemic sclerosis, but it is characterized by significant heterogeneity in patient outcomes. So far, little is known about the influence of anti-U1RNP antibodies on lung outcomes in systemic sclerosis-associated interstitial lung disease patients.
Methods: European Scleroderma Trials and Research group systemic sclerosis patients with radiological-confirmed interstitial lung disease, available %predicted forced vital capacity, and autoantibody status were included.
Objectives: To adapt and evaluate the Coping Strategies Questionnaire-Revised (CSQ-R), designed to assess pain coping, for assessing coping with fatigue in systemic sclerosis (SSc).
Methods: We adapted CSQ-R items for fatigue, and a panel of people with SSc verified content validity. Scleroderma Patient-centred Intervention Network Cohort participants completed the CSQ-R-Fatigue.
Lupus Sci Med
March 2025
Objective: Little is known about the risk of SLE flares associated with hydroxychloroquine (HCQ) reduction or cessation, especially after ophthalmological screening. We analysed the risk of SLE flares after HCQ reduction or discontinuation after detection of early ophthalmological toxicity.
Methods: This study includes all patients with SLE among the 109 included in the prospective PERFOCTAPS Study and treated with HCQ for at least 5 years.
Background: Total lung capacity (TLC) is seldom assessed in the prediction of systemic sclerosis (SSc) disease severity.
Objective: To describe and analyse TLC in SSc.
Methods: We performed a retrospective multicentre study of SSc patients enrolled in the French national SSc cohort with at least one TLC assessment, described patients based on baseline TLC measurements, modelized TLC trajectories in SSc, and associated TLC measures with disease prognosis.
Nat Rev Rheumatol
March 2025
Systemic sclerosis (SSc) remains a challenging and enigmatic systemic autoimmune disease, owing to its complex pathogenesis, clinical and molecular heterogeneity, and the lack of effective disease-modifying treatments. Despite a century of research in SSc, the interconnections among microvascular dysfunction, autoimmune phenomena and tissue fibrosis in SSc remain unclear. The absence of validated biomarkers and reliable animal models complicates diagnosis and treatment, contributing to high morbidity and mortality.
View Article and Find Full Text PDFPurpose: The presentation of ocular sarcoidosis may involve different parts of the eye and/or adnexal tissues. Uveitis is the most common manifestation, and when it involves the posterior segment, it often presents as peripheral multifocal choroiditis. The occurrence of symptomatic occlusive retinal vasculitis (ORV) associated with ocular sarcoidosis is not well described in the literature.
View Article and Find Full Text PDFObjectives: Pulmonary arterial hypertension (PAH) is a rare disease that may be associated with CTD. Anti-fibroblast (AFA) and AECA have been identified in idiopathic and SSc-associated PAH. The aim was to identify autoantibodies discriminating for PAH associated with SLE, MCTD and primary SS, and their target antigens.
View Article and Find Full Text PDFRheumatology (Oxford)
June 2025
Objectives: To describe the characteristics and outcome of patients with the association of large vessel vasculitis (LVV, Takayasu arteritis [TA] or GCA) and IBD.
Methods: An observational, multicentre, retrospective case-control study. Cases were LVV-IBD patients from European countries, whereas controls had isolated LVV (iLVV).
Objectives: Gastroesophageal reflux disease (GERD) is frequent in systemic sclerosis (SSc) and could predict progression of interstitial lung disease (ILD). We aimed to analyse (1) the prevalence of GERD among SSc-ILD patients, (2) its association with disease characteristics and (3) predictive factors for ILD progression in SSc-ILD patients with GERD.
Methods: SSc patients from the EUSTAR database with ILD were included.
Objective: To assess safety of fertility treatments in women with systemic lupus erythematosus (SLE).
Design: Data from the multicentre French observational GR2 (Groupe de Recherche sur la Grossesse et les Maladies Rares) study (2014-ongoing).
Setting: Seventy-six centres in France.
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.
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