Treatment for Rheumatoid Arthritis Associated With Alterations in the Gastrointestinal Microbiota.

Objective: Emerging research suggests that rheumatoid arthritis (RA) is associated with intestinal dysbiosis. This prospective pilot study evaluates changes in intestinal microbial composition in patients with RA initiating treatment with either methotrexate (MTX) or a tumor necrosis factor inhibitor (TNFi).

Methods: Consecutive patients, fulfilling the 2010 American College of Rheumatology/EULAR classification criteria for RA, who started treatment with either MTX or TNFi delivered a stool sample upon initiation of immunosuppression and 3 months later.

Atrioventricular plane displacement and regional function to predict outcome in pulmonary arterial hypertension.

To investigate if left and right atrioventricular plane displacement (AVPD) or regional contributions to SV are prognostic for outcome in patients with pulmonary arterial hypertension (PAH). Seventy-one patients with PAH and 20 sex- and age-matched healthy controls underwent CMR. Myocardial borders and RV insertion points were defined at end diastole and end systole in cine short-axis stacks to compute biventricular volumes, lateral (SV) and septal (SV) contribution to stroke volume.

Anti-Ro52 positivity is associated with progressive interstitial lung disease in systemic sclerosis-an exploratory study.

Background: Interstitial lung disease (ILD) is the most common cause of death in patients with systemic sclerosis (SSc). Prognostic biomarkers are needed to identify SSc-ILD patients at risk for progressive pulmonary fibrosis. This study investigates autoantibodies measured in bronchoalveolar lavage (BAL) fluid and in serum in reference to the clinical disease course of SSc-ILD.

Plasma TRAIL and ANXA1 in diagnosis and prognostication of pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a rare vasculopathy, with high morbidity and mortality. The sensitivity of the current european society of cardiology/european respiratory society (ESC/ERS) risk assessment strategy may be improved by the addition of biomarkers related to PAH pathophysiology. Such plasma-borne biomarkers may also reduce time to diagnosis, if used as diagnostic tools in patients with unclear dyspnea, and in guiding treatment decisions.

Treatment with mycophenolate mofetil is associated with improved nailfold vasculature in systemic sclerosis.

Objective: To investigate the evolution of nailfold capillary density in patients with SSc in relation to immunosuppressive treatment and autoantibodies.

Methods: This was a prospective study cohort. Consecutive newly diagnosed SSc patients were included into this study who, in a retrospective review, had at least two nailfold capillary microscopy measurements performed during the first 48 months of follow-up.

The validity and reliability of the Swedish version of the Satisfaction with appearance scale for individuals with systemic sclerosis.

Background: Systemic sclerosis (SSc) can lead to visible changes in appearance which could generate concerns among patients. Thus, valid questionnaires that capture these concerns are valuable to identify and communicate appearance concerns.

Objective: To determine aspects of the validity and reliability of the Swedish version of the Satisfaction with Appearance scale for individuals with SSc (SWAP-Swe in SSc).

Autoantibodies associated with systemic sclerosis in three autoimmune diseases imprinted by type I interferon gene dysregulation: a comparison across SLE, primary Sjögren's syndrome and systemic sclerosis.

Objective: SLE, primary Sjögren's syndrome (pSS) and systemic sclerosis (SSc) are heterogeneous autoimmune diseases with a dysregulated type I interferon (IFN) system. The diseases often show overlapping clinical manifestations, which may result in diagnostic challenges. We asked to which extent SSc-associated autoantibodies are present in SLE and pSS, and whether these link to serum IFN-α, clinical phenotypes and sex.

Survival in Swedish patients with systemic sclerosis: a nationwide population-based matched cohort study.

Objectives: To conduct the first-ever nationwide, population-based cohort study investigating survival patterns of all patients with incident SSc in Sweden compared with matched individuals from the Swedish general population.

Methods: We used the National Patient Register to identify patients with incident SSc diagnosed between 2004 and 2015 and the Total Population Register to identify comparators (1:5), matched on sex, birth year and residential area. We followed them until death, emigration or the end of 2016.

Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension.

Aims: Repeated risk assessments and treatment patterns over long time are sparsely studied in chronic thromboembolic pulmonary hypertension (CTEPH); thus, we aimed to investigate changes in risk status and treatment patterns in incident patients with CTEPH over a 5 year period.

Methods And Results: Descriptive and explorative study including 311 patients diagnosed with CTEPH 2008-2019 from the Swedish pulmonary hypertension registry, stratified by pulmonary endarterectomy surgery (PEA). Risk and PH-specific treatment were assessed in surgically treated (PEA) and medically treated (non-PEA) patients at diagnosis and up to 5 years follow-up.

Plasma matrix metalloproteinase 2 is associated with severity and mortality in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by vasoconstriction and remodeling of the pulmonary vessels. Risk stratification in PAH could potentially be improved by including novel biomarkers related to PAH pathobiology. We aimed to investigate the relationship between extracellular matrix (ECM)-related proteins, survival, and European Society of Cardiology and European Respiratory Society (ESC/ERS) risk stratification scores in patients with PAH.

Incidence and prevalence of systemic sclerosis in Sweden, 2004-2015, a register-based study.

Objectives: we aim to present an in-depth report of the incidence and prevalence of systemic sclerosis (SSc) in Sweden in a nation-wide register-based study covering the entire Swedish population METHODS: each individual residing in Sweden is given a unique personal identity number. We linked the National Patient Register and the Total Population Register to identify 1) patients with prevalent SSc on 2015-12-31 and 2) patients with incident SSc during the time period 2004-2015 based on ICD-10 codes. We estimated prevalence and incidence overall and stratified on age, sex, and county.

N-Formyl Methionine Peptide-Mediated Neutrophil Activation in Systemic Sclerosis.

Exaggerated neutrophil activation and formation of neutrophil extracellular traps (NETs) are reported in systemic sclerosis (SSc) but its involvement in SSc pathogenesis is not clear. In the present study we assessed markers of neutrophil activation and NET formation in SSc patients in relation to markers of inflammation and disease phenotype. Factors promoting neutrophil activation in SSc remain largely unknown.

Ultrasound and elastography in the assessment of skin involvement in systemic sclerosis: A systematic literature review focusing on validation and standardization - WSF Skin Ultrasound Group.

Objective: To summarize the published evidence in the literature on the role of ultrasound and elastography to assess skin involvement in systemic sclerosis (SSc).

Methods: A systematic literature review (SLR) was performed within the "Skin Ultrasound Working Group" of the World Scleroderma Foundation, according to the Cochrane Handbook. A search was conducted in Pubmed, Cochrane Library and Embase databases from 1/1/1979 to 31/5/2021, using the participants, intervention, comparator and outcomes (PICO) framework.

Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire.

Objectives: Patient-reported outcome measures (PROMs) are important for clinical practice and research. Given the high unmet need, our aim was to develop a comprehensive PROM for systemic sclerosis (SSc), jointly with patient experts.

Methods: This European Alliance of Associations for Rheumatology (EULAR)-endorsed project involved 11 European SSc centres.

Early risk prediction in idiopathic connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment.

Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH). We aimed to investigate differences in clinical characteristics, outcome and performance of the European Society of Cardiology (ESC)/ European Respiratory Society (ERS) risk stratification tool in these patient groups.  This retrospective analysis included incident patients with CTD-PAH (n=197, of which 64 had interstitial lung disease, ILD) or IPAH (n=305) enrolled in the Swedish PAH Register (SPAHR) 2008-2019.

An open-label study to evaluate biomarkers and safety in systemic sclerosis patients treated with paquinimod.

Objectives: To evaluate the changes in disease-related biomarkers and safety of paquinimod, an oral immunomodulatory compound, in patients with systemic sclerosis (SSc).

Methods: In this open-label, single-arm, multicenter study, SSc patients with a rapidly progressive disease received paquinimod for 8 weeks. Blood and skin biopsies were collected at baseline, during treatment, and at follow-up for the analyses of type I interferon (IFN) activity, chemokine (C-C motif) ligand 2 (CCL2), and the number of myofibroblasts.

Predicting mortality during long-term follow-up in pulmonary arterial hypertension.

The European Society of Cardiology (ESC) and European Respiratory Society (ERS) guideline recommendation of comprehensive risk assessments, which classify patients with pulmonary arterial hypertension (PAH) as having low, intermediate or high mortality risk, has not been evaluated during long-term follow-up in a "real-life" clinical setting. We therefore aimed to investigate the utility of risk assessment in a clinical setting for up to 5 years post diagnosis. 386 patients with PAH from the Swedish PAH Registry were included.

Circulating plasma microRNAs in systemic sclerosis-associated pulmonary arterial hypertension.

Objectives: SSc-associated pulmonary arterial hypertension (SSc-APAH) is a late but devastating complication of SSc. Early identification of SSc-APAH may improve survival. We examined the role of circulating miRNAs in SSc-APAH.