NMR spectroscopy compared fat profiles of SSC, healthy blood samples by Margarida Maia, PhD | October 22, 2024

Low levels of high-density lipoprotein (HDL), the so-called “good” cholesterol, are linked to more severe symptoms of systemic sclerosis (SSc) with interstitial lung disease (ILD), a study finds, suggesting that profiling fats in the blood could aid in planning more personalized treatments for the disease.

The study, “Metabolomic signature identifies HDL and apolipoproteins as potential biomarker for systemic sclerosis with interstitial lung disease,” was published in Respiratory Medicine by researchers in Germany.

In SSc, also called scleroderma, overly active immune cells lead to thickened patches of hardened skin and sometimes to scarring in internal organs. When scarring builds up in the lungs, it can cause ILD, where the air sacs and the tissue around them become damaged, making it difficult to breathe.
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What triggers SSc isn’t clear, but circulating fats such as cholesterol may play a role in how the disease develops. Cholesterol is carried in the bloodstream in particles called lipoproteins. One such lipoprotein, HDL, helps remove unused cholesterol from the body. It also protects small blood vessels from damage and prevents blood clots from forming.

Muscle mass, bone density shown to be significant pretransplant predictors
by Steve Bryson, PhD | October 15, 2024

A chest CT scan taken before a lung transplant can help predict survival after the transplant in people with systemic sclerosis (SSc), a new U.S. study suggests.

Muscle mass, bone density, and the volume of the heart and blood vessels were among the “novel image features” of the pretransplant CT that best predicted a patient’s survival, according to the researchers.

Adding CT data to demographic and clinical characteristics improved the accuracy of survival prediction, but CT scans alone could also accurately predict posttransplant survival, the study found.

“Our individualized risk assessment tool can better guide clinicians in choosing and managing patients requiring lung transplant for systemic sclerosis,” the scientists wrote.

Their study, “Predicting post-lung transplant survival in systemic sclerosis using CT-derived features from preoperative chest CT scans,” was published in the journal European Radiology.

Investigating a CT scan of the chest as a possible prediction tool
In SSc, also called scleroderma, a mistaken self-directed immune response drives inflammation and scar tissue buildup, or fibrosis, in the skin and potentially the internal organs.

When the lungs are affected, the scarring makes it more difficult to breathe and can increase the risk of mortality, or death. In severe cases, a lung transplant may be indicated when other therapies have proven to be ineffective.

Lung transplant in SSc accounts for about 0.9% to 1.2% of all lung transplants. This may be due to the disease’s complex, multiorgan nature, which limits the number of clinical centers performing transplants in this patient population.
As such, “there is a pressing need for additional research to understand post-[lung transplant] survival factors and identify those who are suitable for this procedure,” the researchers wrote.
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A CT scan of the chest is routinely performed before a lung transplant to assess the patient’s condition and disease and to monitor the individual’s health status. According to the team, such scans may also contain valuable information that may predict posttransplant complications and survival.

Contrary to earlier finding, TLR8 not present in plasmacytoid dendritic cells
by Margarida Maia, PhD | October 1, 2024

​Activating TLR8, a viral defense mechanism involved in tissue fibrosis (scarring), primes monocytes isolated from the blood of people with systemic sclerosis (SSc) to produce excess amounts of a a molecule called IL-10 that can contribute to the abnormal growth of fibrotic tissue.

The finding opens up possibilities for new therapeutic targets, according to researchers.
“IL-10 could contribute to the fibrotic skin changes that are characteristic of systemic sclerosis,” Theresa Graalmann, PhD, senior study author at the Twincore Center for Experimental and Clinical Infection Research in Hannover, Germany, said in a Twincore press release. “Developing a better molecular understanding of the inflammatory reactions during such a disease is the first step on the road to new and better therapies for the affected patients. However, we cannot yet draw any direct conclusions for treatment because the number of patients is so small.”

The study, “Toll-Like Receptor 8 is Expressed in Monocytes in Contrast to Plasmacytoid Dendritic Cells and Mediates Aberrant Interleukin-10 Responses in Patients With Systemic Sclerosis,” was published as a brief report in Arthritis & Rheumatology.

In SSc, or scleroderma, the immune system mistakenly attacks tissues, leading to fibrosis. When scar tissue accumulates, it causes the skin to become thick and stiff, but can also affect internal organs.
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As a part of the immune system, so-called toll-like receptors (TLRs) help to recognize and fight against infections. In SSc, however, TLRs like TLR8 appear to become overly activated, causing a harmful immune response instead of being protective.