Patients with systemic sclerosis (SSc) frequently develop problems in the heart’s right ventricle. A new study from the Leiden University Medical Center, in the Netherlands, found that both pulmonary fibrosis and pulmonary hypertension were independently linked to the disruption of right ventricular function. Researchers know that several mechanisms might contribute to the development of…
A new model, produced by analyzing 10 different systemic sclerosis (SSc) gene expression data sets, showed that pulmonary fibrosis (PF) in SSc is likely the result of an initial insult activating the interferon signaling pathway. Researchers believe that the uncovered processes might reflect fibrotic processes in all SSc-affected tissues. The research team, led by…
A team of researchers from University College London and Actelion Pharmaceuticals showed that macitentan (Opsumit) can prevent pulmonary arterial hypertension (PAH) in a mouse model of systemic sclerosis (SSc) by preventing heart tissue remodeling and functional changes. The results were presented this week by E. Derrett-Smith at the 4th…
Men with systemic sclerosis (SSc) who develop pulmonary arterial hypertension (PAH) tend to have poorer survival rates in  the first five years after diagnosis, a University of Toronto study reported. Men were also found to develop PAH at an earlier stage in comparison to women, and to suffer a more…
Patients with connective tissue disease or systemic sclerosis associated with pulmonary arterial hypertension (PAH) who are positive for anti-U1 RNP antibodies have more a favorable disease prognosis than patients without these antibodies, a study published in the journal Arthritis & Rheumatology showed. Anti-U1 RNP antibodies are autoantibodies directed at nuclear proteins. They are…
Analyzing data from a genome-wide association study (GWAS), a team led by researchers at the University of Texas McGovern Medical School at Houston showed that systemic sclerosis (SSc)-related interstitial lung disease (ILD) is not genetically linked to idiopathic interstitial pneumonia. SSc-ILD has clinical and radiological similarities to idiopathic interstitial pneumonia, and…
The efficiency of adipose-derived stromal vascular fraction (ADSVF), or cells isolated from fat tissue, to treat ischemia and skin fibrosis in the hands of systemic sclerosis (SSc) patients has been investigation in a small clinical trial. A new study reported on the 12-month follow-up of patients receiving this treatment and…
A review into Wnt pathway signaling as a potential target for systemic sclerosis treatment is optimistic regarding the current status of research and development in the field. The review, “Canonical Wnt signaling in systemic sclerosis,“ was published in the journal Laboratory Investigation by Christina Bergmann and Jörg HW Distler from the …
A new study showed that systemic sclerosis patients with interstitial pneumonia (SSc-ILD) have elevated levels of defensins in their lungs. Defensins are immune molecules with antimicrobial and inflammation regulatory properties. The study, titled “Association of elevated α-defensin levels with interstitial pneumonia in patients with systemic sclerosis,“ was published in the…
Pulmonary hypertension is a common co-morbidity of connective tissue diseases such as scleroderma, occurring in up to 40 percent of patients. Texas-based Reata Pharmaceuticals recently announced that the first patient has enrolled in the LARIAT clinical trial, a study examining the therapeutics effect of bardoxolone methyl in patients with pulmonary…
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