Higher circulating levels of a protein called thymic stromal lymphopoietin, or TSLP, may be linked to a greater risk of digital ulcers — sores in the fingers and toes — in people with systemic sclerosis (SSc), also known as scleroderma, a study revealed. These findings suggest that assessments of TSLP levels could be used to […] The post Blood TSLP protein linked to greater risk of digital ulcers in SSc: Study appeared first on Scleroderma News ..read more

Long-term treatment with Volibris (ambrisentan), marketed as Letairis in the U.S., may help prevent mild pulmonary vascular disease from developing into pulmonary arterial hypertension (PAH) in people with scleroderma, a study suggested. “Early treatment and close follow-up could be beneficial in this high-risk group,” wrote the researchers from Germany and the U.K., who collaborated on […] The post Letairis may prevent PAH linked to scleroderma: Study appeared first on Scleroderma News ..read more

Intravenous immunoglobulin (IVIG) eases skin, muscle and digestive symptoms in people with scleroderma (SSc), particularly when used as an add-on therapy, according to a recent review study. While no improvements were seen in respiratory function, IVIG still led to stabilization of these symptoms. The findings “suggest that IVIG may be effective in treating a range […] The post IVIG effective in easing range of scleroderma symptoms: Review appeared first on Scleroderma News ..read more

Scleroderma patients who have cancer may be treated with radiotherapy without a notable risk of skin and pulmonary worsening, a review study suggests, but around 25% of patients saw severe acute and late toxicities, that is, side effects months after radiation therapy. As such, “individualized assessment, close collaboration between radiation oncologists and rheumatologists, use of […] The post Radiation therapy may be used with caution in scleroderma: Study appeared first on Scleroderma News ..read more

Cells present along the walls of small blood vessels in the lungs, called pericytes, have distinct gene activity in people with systemic sclerosis (SSc)-associated pulmonary fibrosis (PF), according to a new study. Pericytes from SSc patients showed an increased expression, or activity, of genes involved in lung fibrosis (scarring), blood vessel formation, and extracellular matrix organization. […] The post SSc lung cells show gene activity linked to scarring in study appeared first on Scleroderma News ..read more

The European Medicines Agency (EMA) has granted orphan drug status to FT011, an oral therapy candidate that Certa Therapeutics is developing for systemic sclerosis (SSc). The U.S. Food and Drug Administration (FDA) awarded the therapy a similar status last year, followed by fast track designation this year. Orphan drug status is given by both agencies to medicines designed for the treatment of serious or life-threatening rare diseases — those affecting fewer than five in 10,000 people in the European Union or fewer than 200,000 individuals in the U.S. Among its benefits is a period ..read more

Patients with systemic sclerosis (SSc) who have a stem cell transplant see normalized gene activity related to immune function up to 4.5 years after the procedure. That’s according to recent data from samples in the Phase 2/3 SCOT trial (NCT00114530), wherein the potential benefits of a stem cell transplant were compared with those of high-dose Cytoxan (cyclophosphamide) in severe SSc. “The normalization of the SSc [molecular] signature … parallels the clinical benefits of [stem cell transplant] at this time point and provides support for these disease-related pathways as therapeutic targets ..read more

Ferroptosis, a cell death process that can help to regulate uncontrolled cell growth, was suppressed in skin cells from people with systemic sclerosis (SSc), according to recent research. Such suppression appears to be mediated by increased activity of an antioxidant protein called GPX4. Researchers believe that targeting GPX4 to increase ferroptosis could offer a way of preventing the uncontrolled cell growth that drives scar tissue buildup, or fibrosis, in the skin of SSc patients. The study, “Upregulation of GPX4 drives ferroptosis resistance in scleroderma skin fibroblasts,” was published ..read more

CONQUEST, a platform clinical trial launched by the Scleroderma Research Foundation (SRF), is now enrolling people with interstitial lung disease associated with systemic scleroderma (SSc-ILD) at centers in the U.S. The study is analyzing two investigational therapies using a master protocol to compare both treatments with a shared placebo group. It currently includes investigational therapies developed by Sanofi and Boehringer Ingelheim for SSc-ILD, but new medications may be added or removed. The main goal of trials like CONQUEST is to identify treatments that are worthy of advancing from e ..read more

Treatment with Letairis (ambrisentan) — approved to treat pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis (SSc) — may help prevent the development of PAH, or high blood pressure in the arteries of the lungs, in people with SSc. That’s according to new data from SSc patients who completed the Phase 2 EDITA clinical trial (NCT02290613), which tested Letairis against a placebo, and who then continued their assigned regimen over a longer period. These results, along with positive data from the Phase 2 AST-MOMA trial (NCT01895244) — which ..read more