MD News and Information

Systematic Autoantigen Analysis Identifies a Distinct Subtype of Scleroderma with Coincident Cancer

Author: George J. Xu, Ami A. Shahf, Mamie Z. Li, Qikai Xu, Antony Rosen, Livia Casciola-Rosen, and Stephen J. Elledge
Date Published: November-2016
Source: PNAS

In this study, we created a barcoded whole-genome ORF mRNA display library and combined it with phage-immunoprecipitation sequencing to look for autoantibodies in sera from patients with scleroderma who also had coincident cancer without a known autoantibody biomarker. Using these two technologies, we found that 25% of these patients had autoantibodies to RNA Binding Region Containing 3 (RNPC3) and multiple other components of the minor spliceosome. There was evidence of intra- and intermolecular epitope spreading within RNPC3 and the complex. These combined technologies are highly effective for rapidly discovering autoantibodies in patient subgroups, which will be useful tools for patient stratification and discovery of pathogenic pathways.

 

Rituximab Effective Long-Term for Systemic Sclerosis

Author: Pauline Anderson
Date Published: November-2016
Source: MedPage Today

New research provides additional evidence that rituximab, a B-cell depletion therapy, improves lung fibrosis and reduces skin thickening in patients with systemic sclerosis (SSc).

Over 7 years, pulmonary function was stabilized or improved in SSc patients with interstitial lung disease receiving rituximab, and the drug also helped resolve skin thickening in these patients. As well, the study showed that cessation of rituximab therapy was associated with a decline in pulmonary function, and that the drug had an acceptable safety profile.

 

New Study Suggests Way to Slow Skin Fibrosis in Scleroderma

Author: HSS
Date Published: November-2016
Source: Press Release

The prognosis for patients diagnosed with scleroderma - an autoimmune disease characterized by fibrosis of the skin - is not typically a rosy one. With limited treatment options available, those suffering from the disorder can face disabling hardening and tightening of their skin. Scleroderma can also affect the blood vessels, lungs and other internal organs.

 

Many Systemic Sclerosis Patients with Raynaud’s Syndrome Soon Develop Other Conditions

Author: Joana Fernandes, PHD
Date Published: October-2016
Source: Scleroderma News

Patients with systemic sclerosis and Raynaud’s syndrome have a high risk of developing other organ complications within two years after the onset of Raynaud’s, according to a study published in the journal PLoS One. The authors reported that these complications mainly occur in the skin, gastrointestinal tract, lungs, heart, kidneys and prostate. The study, “Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study,” was conducted by Veronika Jaeger and her colleagues from the University Hospital Basel in Switzerland.

 

PAH Medications, Tracleer and Opsumit, Seen to Block Fibrosis in Systemic Sclerosis in Early Study

Author: Joana Fernandes, PHD
Date Published: October-2016
Source: Scleroderma News

Two approved treatments for pulmonary arterial hypertension — Tracleer (bosentan) and Opsumit (macitentan) — can block a molecular pathway that promotes fibrosis in systemic sclerosis (SSc), and may be a potential therapy for these patients as well, according to an early study. The study, “Bosentan And Macitentan Prevent The Endothelial-To-Mesenchymal Transition (Endomt) In Systemic Sclerosis: In Vitro Study,” was published in the journal Arthritis Research & Therapy.

 

Targeted Therapy for Scleroderma Fibrosis

Author: Sara R. Schoenfeld, MD, & Flavia V. Castelino, MD
Date Published: October-2016
Source: The Rheumatologist

Scleroderma, or systemic sclerosis (SSc), is an autoimmune disease characterized by vasculopathy and fibrosis. Although relatively rare, with a prevalence in North America of approximately 300 per 1 million people, SSc is associated with significant morbidity and high rates of mortality.1 Patients with scleroderma have four times greater mortality than age- and sex-matched controls, with the majority of deaths related to interstitial lung disease (ILD) and pulmonary hypertension (PH).2

 
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