About 105,410 results

Histopathological Patterns of Muscle Involvement and Clinical Correlations in a Retrospective Cohort of Scleroderma Patients
Noemie Le Gouellec, Louai Zaidan, Eric Hachulla et. al.

Nov 14th, 2022 - Background/Purpose: Muscle involvement in systemic sclerosis (SSc) has been shown to be heterogeneous. Histopathological pattern seems to be associated with prognosis, but studies evaluating the correlations between muscle involvement subtypes, clinical presentation and outcomes are scarce. The aim of this study was to evaluate the prognostic value of histopathological muscular type in SSc. Met...

Focused Clinical Correlation of Autoantibody Testing in Juvenile Systemic Sclerosis and Localized Scleroderma Using the Euroimmun™ Scleroderma Disease Profile
Jonathan Li, Emily Mirizio, Katherine Buhler et. al.

Nov 14th, 2022 - Background/Purpose: Commercially available autoantibody (AAb) panels are developed for adult rheumatological diseases; application to pediatric disease has not been established. As a pediatric scleroderma referral center and clinic, one of our main interests is to examine the properties of the Euroimmun™ Scleroderma Disease Profile in juvenile-onset scleroderma. These panels may be useful for p...

Nailfold Videocapillaroscopic Abnormalities Correlate with Disease Activity Measures and Cutaneous Damage in Patients with Idiopathic Inflammatory Myopathies
Diane Zisa, Aliza Bloostein, Deanna Jannat-Khah et. al.

Nov 14th, 2022 - Background/Purpose: Nailfold videocapillaroscopy (NVC) is a non-invasive tool used to evaluate the microcirculation of patients with connective tissue diseases (CTDs). A specific combination of NVC abnormalities, known as the “scleroderma pattern,” has been well-described in patients with systemic sclerosis and demonstrated to have prognostic significance. The significance of these changes in p...

Autoantibodies Are Common in Patients with Idiopathic Interstitial Lung Disease Suggesting a High Prevalence of Undiagnosed Autoimmune Connective Tissue Disease
Sarah Tansley, Caroline Cotton, Fionnuala McMorrow et. al.

Nov 14th, 2022 - Background/Purpose: In some patients, interstitial lung disease (ILD) may be the dominant or even sole overt manifestation of an otherwise unrecognised autoimmune connective tissue disease (CTD). Accurate diagnosis can be challenging given considerable overlap of the clinical, radiological and histological disease features. Distinguishing CTD related ILD from idiopathic ILD, enables appropriate...

Clinical Outcomes in an Observational Retrospective Cohort of Patients with Connective Tissue Disease-associated Interstitial Lung Disease (CTD-ILD) Who Taper Immunosuppressive Therapy
Paul Dellaripa, Paul Hoover and Tracy Doyle, Brigham and Women's Hospital et. al.

Nov 14th, 2022 - Background/Purpose: Patients with CTD-ILD are often treated with a combination of corticosteroids and steroid sparing agents to limit the progression of the inflammatory response in the lung and prevent remodeling that can lead to fibrosis. While tapering regimens for steroids are part of the standard of care in CTD-ILD, there is no significant experience on outcomes in patients who taper stero...

Impact of a Fatigue and Energy Management Program for Persons with Systemic Sclerosis (FAME-iSS): A Pilot Study
Janet Poole, Deirdre Connolly, Jessica Salazar3 and Kristine Carandang et. al.

Nov 14th, 2022 - Background/Purpose: Fatigue is one of the most prevalent and disabling symptom reported by people with systemic sclerosis (SSc). No fatigue-specific programs exist for people with SSc despite the negative impact on daily life activities. Fatigue and Activity Management Education (FAME-ISS) is a six-week virtual program led by occupational therapists that was adapted for SSc. Topics covered fact...

Gastroesophageal Reflux Disease in an Australian Scleroderma Cohort – Associations and Effect of Treatment
Alannah Quinlivan, Dennis Neuen, Dylan Hansen et. al.

Nov 14th, 2022 - Background/Purpose: To investigate the association between gastroesophageal reflux disease (GORD) and interstitial lung disease (ILD) and determine the effect of GORD treatment on survival in Australian scleroderma (SSc) patients. Methods: One thousand, six hundred and forty consecutive SSc patients from the Australian Scleroderma Cohort Study (ASCS) were included. Patients were considered to h...

Alterations of Nutritional Status of Scleroderma Patients and Associations with Disease-specific Features
Sabína Oreská, Hana Storkanova, Maja Spiritovic et. al.

Nov 14th, 2022 - Background/Purpose: Systemic sclerosis (SSc) is characterized by skin and organ involvement and chronic disease course. In particular, involvement of the gastrointestinal tract and systemic inflammation can have a negative impact on nutritional status. The aim of this study was to assess the differences in the nutritional status of SSc patients and healthy controls (HC) and the association with...

A Novel Pharmaco-Interventional Approach for the Treatment of Refractory Digital Ischemia in Systemic Sclerosis – Percutaneous Revascularization Combined with Intravenous Vasodilatory Therapy
Lily Romero Karam, Kevin Honan, Salman Arain1 and Maureen Mayes et. al.

Nov 14th, 2022 - Background/Purpose: The combination of Raynaud´s Phenomenon (RP) and vasculopathy, characterized by intimal proliferation and fibrosis, endothelial dysfunction, and smooth-muscle activation, can lead to severe digital ischemia in patients with systemic sclerosis (SSc). Despite standard treatments, a subset of patients sustain prolonged disability and experience tissue loss including digital amp...

Evaluation of Functional Disability over Time in the Leiden Systemic Sclerosis (CCISS) Cohort
Sophie Liem, Saad Ahmed, Tom Huizinga et. al.

Nov 14th, 2022 - Background/Purpose: Functional disability in patients with systemic sclerosis (SSc) greatly influences quality of life. In SSc, the goal of treatment is to decrease disease burden and improve HRQoL. Therefore, it is important to gain insight in the longitudinal course of functional disability in SSc patients from cohorts reflecting the complete SSc spectrum, and especially identify patients who...

The Role of CCL21 in Serum Samples from Systemic Sclerosis Patients
Henriette Didriksen, Øyvind Molberg, Adi Mehta et. al.

Nov 14th, 2022 - Background/Purpose: Chemokines appear to be important for the pathogenesis of autoimmune diseases. Humans may generate antibodies targeting chemokines leading to inhibition of signaling and driving disease phenotypes. We have reported CCL21 as a promising serum marker for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) by applied enzyme linked immunosorbent analysis (ELISA). A...

Autoantibodies in Patients with Early Systemic Sclerosis in the Collaborative National Quality and Efficacy Registry
Alyssa Bosso, Shervin Assassi, Tracy Frech et. al.

Nov 14th, 2022 - Background/Purpose: We sought to describe the autoantibody profile of SSc patients with early disease and examine the clinical, laboratory and prognostic features associated with these antibody subsets. Methods: Study participants were from the Collaborative National Quality and Efficacy Registry (CONQUER), a multicenter US-based registry of SSc patients within 5 years (median 2.2-2.8 years) of...

An Exploratory Phosphoproteomic and Immunohistochemical Analysis of Receptor Tyrosine Kinases and Downstream Signaling Pathways in Systemic Sclerosis Skin
Elizabeth Bundschuh, Rebecca Fine, Miruna Carnaru et. al.

Nov 14th, 2022 - Background/Purpose: Receptor tyrosine kinases (RTK) such as epidermal growth factor receptor (EGFR) and others including PDGFRs, FGFRs, and VEGFRs are implicated in systemic sclerosis (SSc) skin fibrosis. We previously identified a subpopulation of fibroblasts that express EGFR and drive SSc skin fibrosis in a multicellular circuit. This study sheds light upon molecular pathways downstream of R...

Validation of Ranked Composite Important Difference (RCID) Score in Early Diffuse Cutaneous Systemic Sclerosis
Francesco Del Galdo, Suiyuan Huang, Lesley-Anne Bissell et. al.

Nov 14th, 2022 - Background/Purpose: The Ranked Composite Important Difference (RCID) in diffuse cutaneous Systemic Sclerosis ( dcSSc) is a clinically and patient meaningful composite score for outcome of dcSSc developed as anchor of the ACR Composite Response Index in SSc (ACR-CRISS) following OmerACT methodology. The score assigns a specific weight to Organ Failure, and Minimal Clinically important difference...

Serum IFN Score Predicts Long Term Outcome in Limited Cutaneous Systemic Sclerosis
Ranjitha Karanth, Giuseppina Abignano, Vishal Kakkar et. al.

Nov 14th, 2022 - Background/Purpose: Type-I Interferon pathway activation has been associated with severity and progression of diffuse cutaneous SSc (dcSSc). The role of type-I Interferons (IFNs) in limited cutaneous systemic sclerosis (lcSSc) has not yet been explored. Here we aimed to determine the value of Type I IFN activation in stratifying for clinical outcome in lcSSc as assessed by a novel composite end...

Pulmonary Function in Patients Diagnosed of Early Systemic Sclerosis: 10 Years-Experience
Samuel Leal Rodriguez, Francisco Miguel Ortiz Sanjuan, José Ivorra Cortés et. al.

Nov 14th, 2022 - Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc), often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension. Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from ...

Evaluation of the Distribution of Haemodynamic Parameters and Prognostic Impact in a Cohort of Patients with Systemic Sclerosis
Zoe Brown, Dylan Hansen, Wendy Stevens et. al.

Nov 14th, 2022 - Background/Purpose: Scleroderma (SSc) is a complex multisystem autoimmune disease, characterised by vasculopathy and fibrosis of skin and organs. Involvement of the cardiovascular system occurs frequently and is associated with significant morbidity and mortality. Pulmonary hypertension (PH) may result from a number of causes in SSc, including pulmonary vasculopathy or as a consequence of inter...

BNT162b2 mRNA Vaccine Against Covid 19, Humoral Response, Safety and Efficacy in Scleroderma Patients
Yolanda Braun-Moscovici, Marielle Kaplan, Maya Braun et. al.

Nov 14th, 2022 - Background/Purpose: In the begining of pandemic and till spring 2021 there were no clinical and laboratory data regarding sefety and efficacy of mRNA vaccines against Covid 19 in patients (pts) with systemic sclerosis (SSc), particularly treated with DMARDs (conventional [cs] and biological [bDMARD]). Our aims were to assess the humoral response to 2 doses of mRNA vaccine against Covid 19 and t...

Novel Tissue Turnover Blood Markers Are Associated with Pulmonary Arterial Hypertension and NYHA Stages in Patients with Systemic Sclerosis
Signe Holm Nielsen, Sine Søndergaard Korsholm, Axel Diederichsen et. al.

Nov 14th, 2022 - Background/Purpose: Systemic sclerosis (SSc) is characterized by vasculopathy and fibrosis of the skin and internal organs. Cardiac involvement, pulmonary arterial hypertension (PAH), and interstitial lung disease (ILD) are associated with a poor prognosis. They can develop clinically silent and be difficult to detect. Novel tissue turnover blood markers hold the potential to detect the manifes...

Influence of Immunosuppressive Therapy on Gastrointestinal Symptoms in Patients with Systemic Sclerosis
Lea Stamm, Alexandru Garaiman, Norina Zampatti et. al.

Nov 14th, 2022 - Background/Purpose: The gastrointestinal (GI) tract is frequently affected in systemic sclerosis (SSc), leading to considerable morbidity and even mortality. While important progress has been made in the last years regarding treatment of SSc, there is no disease-modifying treatment available for SSc-related GI involvement. In this observational study of unselected patients with SSc, we aimed to...