Scleroderma

Clinical Features Identified for Localized Scleroderma in Children

Clinical Features Identified for Localized Scleroderma in Children

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Researchers conducted a multicenter prospective study to identify clinical features characteristic of localized scleroderma in children.

Depression Prevalent Among Individuals With Systemic Sclerosis

Depression Prevalent Among Individuals With Systemic Sclerosis

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Depression is prevalent in a considerable proportion of patients with systemic sclerosis.

Ultrasonographic Synovial Involvement Frequent in Systemic Sclerosis

Ultrasonographic Synovial Involvement Frequent in Systemic Sclerosis

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Ultrasonographic synovial involvement is frequent in patients with systemic sclerosis.

Gene Therapy for Localized Scleroderma Gets FDA's Fast Track Designation

Gene Therapy for Localized Scleroderma Gets FDA's Fast Track Designation

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Clinical enrollment for an open-label, single-arm Phase 1/2 trial of FCX-013 was initiated in August 2018.

Rituximab Safe and Efficacious Alternative Treatment for Scleroderma

Rituximab Safe and Efficacious Alternative Treatment for Scleroderma

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Rituximab is a safe and effective alternative to cyclophosphamide in managing the progression of scleroderma and subsequent development of interstitial lung disease.

Older Age Associated With Greater Cardiac Involvement in Early Systemic Sclerosis

Older Age Associated With Greater Cardiac Involvement in Early Systemic Sclerosis

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In patients in the early stages of systemic sclerosis, older patients had more severe disease with greater cardiac involvement.

Risk Factors for Scleroderma Renal Crisis in Systemic Sclerosis

Risk Factors for Scleroderma Renal Crisis in Systemic Sclerosis

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Researchers identify risk factors for scleroderma renal crisis in patients with systemic sclerosis.

Strong Reproducibility for 6-Minute Walk Test in Systemic Sclerosis

Strong Reproducibility for 6-Minute Walk Test in Systemic Sclerosis

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Investigators assessed the reproducibility and the utility of the 6-minute walk test in systemic sclerosis.

Little Data Available for Small Intestinal Bacterial Overgrowth Treatment in Systemic Sclerosis

Little Data Available for Small Intestinal Bacterial Overgrowth Treatment in Systemic Sclerosis

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For some patients with systemic sclerosis, antibiotics can eradicate small intestinal bacterial overgrowth.

SPAR Model May Predict Mild Interstitial Lung Disease in Systemic Sclerosis

SPAR Model May Predict Mild Interstitial Lung Disease in Systemic Sclerosis

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The SPAR prediction model might be helpful for the risk stratification of mild ILD in patients with SSc in clinical practice.

PAH in Systemic Sclerosis Increases Risk for Early Mortality

PAH in Systemic Sclerosis Increases Risk for Early Mortality

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The majority of deaths in patients with systemic sclerosis-associated pulmonary arterial hypertension occurred within 4 years of diagnosis.

Sildenafil, Bosentan Combination vs Sildenafil Monotherapy in SSc-Associated PAH

Sildenafil, Bosentan Combination vs Sildenafil Monotherapy in SSc-Associated PAH

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There was no significant difference in efficacy between sildenafil and bosentan initial combination therapy and sildenafil monotherapy in patients with systemic sclerosis-related pulmonary arterial hypertension.

Smoking Not Associated With Systemic Sclerosis-Specific Cutaneous, Pulmonary Manifestations

Smoking Not Associated With Systemic Sclerosis-Specific Cutaneous, Pulmonary Manifestations

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Smoking status in patients with systemic sclerosis was not significantly associated with systemic sclerosis-specific cutaneous or pulmonary changes.

PAH-Associated Connective Tissue Diseases: A Case for Separating SSc, SLE

PAH-Associated Connective Tissue Diseases: A Case for Separating SSc, SLE

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Recent evidence suggests that systemic sclerosis and SLE in pulmonary arterial hypertension should be considered separate diseases.

LPA1 Receptor Antagonist SAR100842 Safe, Well Tolerated in Systemic Sclerosis

LPA1 Receptor Antagonist SAR100842 Safe, Well Tolerated in Systemic Sclerosis

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The investigational drug SAR100842 was safe, moderately effective, and well tolerated in patients with early diffuse cutaneous systemic sclerosis.

Expert Insight: Detecting Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis

Expert Insight: Detecting Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis

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Although pulmonary arterial hypertension occurs in a minority of patients with systemic sclerosis, clinicians must be attentive to it, as it can emerge at any point over the course of the disease.

Scleroderma Phenotype, Autoantibodies May Identify Patients at Risk for Cancer

Scleroderma Phenotype, Autoantibodies May Identify Patients at Risk for Cancer

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Stratification by clinical features and autoantibody response identify scleroderma subgroups with distinct risks for cancer.

Independent Predictors of VTE Identified in Systemic Sclerosis

Independent Predictors of VTE Identified in Systemic Sclerosis

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Researchers evaluated the prevalence, risk factors, and effect of venous thromboembolism on survival in systemic sclerosis.

Predicting Skin Score Changes in Early Diffuse Systemic Sclerosis

Predicting Skin Score Changes in Early Diffuse Systemic Sclerosis

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Two predictive models for progressive skin thickening in patients with early diffuse cutaneous systemic sclerosis were developed to use in clinical practice and to improve recruitment in clinical trials.

Social Anxiety in Systemic Sclerosis: Comparing Patient Assessment Tools

Social Anxiety in Systemic Sclerosis: Comparing Patient Assessment Tools

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Investigators assessed 3 versions of the Brief Fear of Negative Evaluation scale in patients with systemic sclerosis.

Scleroderma and Breast Cancer: Examining Outcomes of Radiation Therapy

Scleroderma and Breast Cancer: Examining Outcomes of Radiation Therapy

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Researchers sought to determine the prevalence of radiation complications in patients with systemic scleroderma and breast cancer.

Stem Cell Transplant May Benefit Patients With Systemic Sclerosis

Stem Cell Transplant May Benefit Patients With Systemic Sclerosis

Patients who underwent autologous hematopoietic stem cell transplant for systemic sclerosis saw improvements in quality of life.

PAH Incidence in Systemic Sclerosis: Does Algorithm Use Affect Reported Incidence?

PAH Incidence in Systemic Sclerosis: Does Algorithm Use Affect Reported Incidence?

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Use of the DETECT algorithm did not change incidence of PAH, but led to an increased frequency of cases of borderline pulmonary hypertension.

Early Scleroderma Disability May Be Affected by Skin Thickening, Poor Hand Function

Early Scleroderma Disability May Be Affected by Skin Thickening, Poor Hand Function

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These results likely indicate the importance of skin thickening in contributing to impairment of hand function.

Patients With Early SSc-PAH Development Have Substantial Rate of Disease Progression

Patients With Early SSc-PAH Development Have Substantial Rate of Disease Progression

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Patients with systemic sclerosis and early-diagnosed pulmonary arterial hypertension had disease progression over a relatively short period of time.

Predicting Mortality in Systemic Sclerosis-PAH With CT Signs of Veno-Occlusive Disease

Predicting Mortality in Systemic Sclerosis-PAH With CT Signs of Veno-Occlusive Disease

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A blinded analysis sought to determine whether CT signs of pulmonary veno-occlusive disease are common and predictive of mortality in patients with systemic sclerosis and pulmonary arterial hypertension.

Assessment of Life Expectancy in Scleroderma-Associated Pulmonary Arterial Hypertension

Assessment of Life Expectancy in Scleroderma-Associated Pulmonary Arterial Hypertension

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Patients with scleroderma-associated isolated pulmonary arterial hypertension (SSc-PAH) have a slightly higher mortality risk than patients SSc-PAH with concomitant interstitial lung disease.

Pulmonary Arterial Hypertension in Systemic Sclerosis: Swift Diagnosis and Effective Treatment

Pulmonary Arterial Hypertension in Systemic Sclerosis: Swift Diagnosis and Effective Treatment

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Rheumatology specialists should be aware of the clinical features of SSc-PAH, as well as the tools available to facilitate a prompt, accurate diagnosis.

Key Hemodynamic Differences Noted With Scleroderma-Associated PAH vs Idiopathic PAH

Key Hemodynamic Differences Noted With Scleroderma-Associated PAH vs Idiopathic PAH

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Patients with systemic sclerosis-associated pulmonary arterial hypertension display important differences in hemodynamic and pulmonary function parameters compared with patients with idiopathic PAH.

Systemic Sclerosis: Capillaroscopic and Ultrasonographic Evaluations

Systemic Sclerosis: Capillaroscopic and Ultrasonographic Evaluations

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Microvascular damage evaluated using nailfold capillaroscopy and macrovascular features that include ulnar artery occlusion, assessed per hand power Doppler ultrasonography, have demonstrated associations with the main digital manifestations of systemic sclerosis.

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