Researchers used machine learning algorithms to analyze data by Margarida Maia, PhD | February 4, 2025 The CCL2 gene, which codes for a signaling protein of the same name, is “a common characteristic gene” of both systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF), a serious complication of SSc that causes the lungs to become inflamed and scarred, according to a study by researchers in China.
“We identified CCL2 as a common biomarker from IPF and SSc, revealing the common mechanism of these two diseases,” the researchers wrote in “Common biomarkers of idiopathic pulmonary fibrosis and systemic sclerosis based on WGCNA and machine learning,” published in the journal Scientific Reports. According to the researchers, their study provides “clues for the study of the treatment and mechanism of these two diseases.” However, these findings need to be confirmed by lab and clinical testing, given that the study “used data from a public database and lacked support from clinical data,” in addition to other limitations, the team noted. The researchers used machine learning algorithms, a type of artificial intelligence, to analyze the data from that database. Sex differences uncovered in US study for protein tied to wound healing by Andrea Lobo, PhD | January 28, 2025 Autotaxin, a protein involved in wound healing and scarring, or fibrosis, was found at higher levels in women with systemic sclerosis-associated interstitial lung disease (SSc-ILD) than in men with the condition, per a new U.S. study.
According to the researchers, “this study is the first to report sex-specific … protein differences in patients with SSc-ILD.” Overall, 40 proteins were found to be present at different levels in men and women with SSc-ILD, with the team noting that “none of these proteins have been previously associated with sex-related hormones.” However, only autotaxin was shown to be present at “significantly different” levels between the sexes in a subsequent analysis, the researchers noted. “These proteins could influence disease progression and treatment response and underscore the importance of personalized therapeutic strategies and further research into sex-related molecular pathways in SSc-ILD,” the team wrote. Their study, “An exploratory analysis of differences in serum protein expression by sex in patients with systemic sclerosis associated interstitial lung disease,” was published in the journal BMC Pulmonary Medicine. Protein may serve as tool for evaluating long-term dialysis risk, said researchers by Katherine Poinsatte | January 21, 2025 Scleroderma (SSc) patients who’ve had a scleroderma renal crisis, a life-threatening complication of SSc, have higher levels of placental growth factor (PIGF), protein that stimulates blood vessel growth, in their blood than those who haven’t had one, a study in France shows.
Among French patients who’d had a scleroderma renal crisis, higher PIGF levels were also found in those who reached end-stage kidney failure compared with SSc patients who didn’t need dialysis, which is a treatment to clean the blood. “Serum [blood] PlGF may identify the risk of [scleroderma renal crisis] occurrence among SSc patients with a good specificity and represents a potential tool for long-term dialysis risk evaluation,” the researchers wrote. The study, “Pilot Study of diagnostic performances of vascular biomarkers soluble fms-like tyrosine kinase and placental growth factor in scleroderma renal crisis,” was published in Kidney International Reports. In a scleroderma renal crisis, abrupt blood pressure changes and acute kidney failure augurs a risk of of chronic kidney disease and dialysis. One of the few clinically measurable risk factors for developing scleroderma renal crisis is the presence of antibodies against RNA polymerase III. No biomarker has been able to help predict kidney prognosis after a scleroderma renal crisis, however. PIGF has been found at higher levels in SSc patients with pulmonary hypertension, a condition where blood pressure in the lungs is abnormally high, compared with people with SSc but without pulmonary hypertension. Classifying disease by skin symptoms was not associated with either by Katherine Poinsatte | January 14, 2025 Organ involvement and outcomes among people with systemic sclerosis (SSc) are strongly associated with seven specific self-reactive antibodies, a French study has found.
Among their findings, researchers identified antibodies that targets RNA polymerase III as strongly associated with scleroderma renal crisis, a rare and severe manifestation of SSc marked by high blood pressure, and kidney and heart failure. Classification by skin symptoms wasn’t associated with survival or organ involvement. “The systematic and accurate determination of [specific self-reactive antibodies] at diagnosis could help clinicians to better stratify the individual risk of developing SSc complications and personalize monitoring,” the researchers wrote. The study, “Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients,” was published in RMD Open. SSc, also called systemic sclerosis, features excessive scarring in the skin and may also occur in the heart, kidney, lungs, and gastrointestinal tract. Patients are divided into two main subtypes, limited and diffuse, based on the extent of their skin symptoms. Limited SSc patients may have skin symptoms on the face, arms, hands, and fingers, while people with diffuse SSc have widespread skin fibrosis, or scarring, and a higher risk of internal organ involvement. The skin-based subtypes have traditionally predicted organ involvement and mortality. However, given that not all SSc patients exhibit skin fibrosis and that serious lung involvement may occur in both subtypes, the researchers believe better ways of predicting organ involvement and disease outcomes are needed. “The skin phenotype is not a sufficient and reliable factor to formally predict organ damage and disease complications for clinicians,” the researchers wrote. The immune system of people with SSc makes self-reactive antibodies that mistakenly attack healthy tissue. While several specific autoantibodies have been linked to the disease and particular organ involvement, the exact biological consequences of self-reactive antibodies are poorly understood. Zura Bio has launched a Phase 2 trial to evaluate the safety and efficacy of tibulizumab, its experimental dual pathway antibody to treat systemic sclerosis (SSc).
The TibuSURE trial is expected to enroll as many as 80 adults with early diffuse cutaneous SSc. They will receive tibulizumab or a placebo for 24 weeks (about six months). This will be followed by an open-label extension of 28 weeks, in which all participants will receive the treatment. “We believe tibulizumab’s dual-pathway approach holds the potential to be best-in-class, aiming to provide deeper efficacy and greater benefits for patients affected by this life-threatening autoimmune disease,” Kiran Nistala, PhD, Zura Bio’s chief medical officer and head of development, said in a company press release. “The initiation of the TibuSURE study marks a significant milestone in addressing certain urgent, unmet needs of this patient population and advancing our mission to improve the lives of those affected by autoimmune and inflammatory conditions.” SSc is an autoimmune disease characterized by inflammation and fibrosis, or the accumulation of scar tissue in the skin and internal organs, such as the lungs, heart, kidneys, and digestive tract. Two therapies (Ofev and Actemra) are available to treat severe lung complications associated with the disease, but there is no approved treatment for multiple organ manifestations. GI symptoms are my constant unwanted companion by Tomisa Starr | December 18, 2024 I think of scleroderma as my constant companion — one that tries to keep me from having a life of my own. Whenever I want to do anything outside of my home or lead a productive life, scleroderma always seems to hold me back. It’s like the friend I can’t take anywhere because they’re always doing things to embarrass me.
Of all the problems caused by my scleroderma, gastrointestinal (GI) issues are the worst. These are my most embarrassing and painful symptoms. Stomach noises may be embarrassing, but they’re a sign that the GI tract is working as it should. These sounds mean that the food we’ve eaten is being propelled through our digestive system and is on its way to the intestines. In the past, I’d occasionally hear my stomach churning whenever I was especially hungry, and I could hear my intestines working to expel gas from my body. If there’s anything worse than the dismay most people experience when their stomach makes embarrassing noises, it’s when those noises suddenly stop and you realize that something is wrong. How Superman and Christopher Reeve have inspired me over time by Tomisa Starr | December 11, 2024 Years before my scleroderma diagnosis, I was a fighter. I was born almost three months premature and came into this world fighting. One of my maternal grandmother’s friends asked her if I would live. “She’s too ornery not to,” my grandmother said. Even then, she knew I was a fighter.
I also had quite the temper. I discovered Superman comics (they came in many titles) at age 6. Superman inspired me because he’s big, strong, and powerful — physically, everything I wasn’t. Superman protected human beings. He never retaliated against them, no matter what they’d done. He fought injustice, and he always stood up for what was good. When I watched the news with my parents and grandparents, I saw a lot of injustice in the world. I wanted to be like Superman and fight it, but I was only 6. Since I couldn’t save the world, I decided to make a positive change in myself by working on my temper. Superman inspired me because he never got angry. Smoking, higher BMI linked to more fatigue; older age, being male to less by Andrea Lobo, PhD | December 3, 2024 Fatigue levels in people with scleroderma, also known as systemic sclerosis or SSc, are tied to several social, demographic, lifestyle, and disease-related factors, according to a study by a multinational team of researchers.
Smoking, higher body mass index (BMI) -- a measure of body fat based on height and weight -- pain, gastrointestinal involvement, and lung disease were among the factors associated with more fatigue. In contrast, older age and male sex were linked to lower fatigue scores. “Some factors that we have identified may be modifiable using generic interventions, including BMI and current smoking; as with any patients, people with SSc should be advised and supported to maintain a healthy lifestyle,” the researchers wrote, noting, however, that “in people with SSc, fatigue scores were substantially higher than in the general population.” The team also called on clinicians to focus on pain when treating scleroderma patients, and seek ways to lessen its impact. “Given the substantial association of pain with fatigue in our study, healthcare professionals should work with patients to identify aspects of [the] disease that are causing pain and attempt to address them,” the researchers wrote. The study, “Fatigue levels and associated factors in systemic sclerosis: a cross-sectional study of 2385 SPIN Cohort participants,” was published in the journal Rheumatology. Therapy also being developed for other autoimmune diseases, cancer by Steve Bryson, PhD | November 26, 2024 Soquelitinib, Corvus Pharmaceuticals’ immune-modulating investigational oral treatment for autoimmune diseases and certain cancers, reduced the signs and symptoms of lung disease caused by systemic sclerosis (SSc), according to new preclinical data.
Designed to selectively block ITK, an enzyme predominantly found in immune T-cells, soquelitinib suppresses Th2 cells — a type of immune helper T-cells whose activation drives the development of many autoimmune and allergic diseases. At the same time, ITK inhibition promotes the growth of Th1-type helper T-cells, which are required for immunity to infections and tumors. The treatment’s goal is to prevent lung damage, inflammation, and high blood pressure related to SSc. It’s also being investigated in other autoimmune diseases and cancers associated with T-cells. “We continue to build evidence that selective ITK inhibition can modulate immune responses for a wide range of immune diseases,” Richard A. Miller, MD, co-founder, president, and CEO of Corvus, said in a company press release. Yannick Allanore, MD, PhD, a professor of rheumatology at Université Paris Cité in France, presented the preclinical data as a poster at the American College of Rheumatology Convergence 2024, held in earlier this month in Washington, D.C. Researchers sequence lung fibroblasts to test hypothesis by Steve Bryson, PhD | November 19, 2024 Cells derived from people with systemic sclerosis (SSc) had widespread DNA mutations compared with healthy cells, which may explain the higher risk of cancer in this patient population, according to a study.
Changes included mutational patterns found solely in the genomes of certain cancers, as well as changes in one or two DNA building blocks, insertions and deletions of DNA segments, and alterations in whole chromosomes. The study, “Widespread mutagenesis and chromosomal instability shape somatic genomes in systemic sclerosis,” was published in Nature Communications. SSc, or scleroderma, is an autoimmune disorder that affects the skin and connective tissue. It’s marked by the build-up of scar tissue in the skin and potentially various organs, including the heart and blood vessels, as well as the lungs, stomach, and kidneys. Studies suggest that the incidence of cancer in people with SSc is higher than in the general population, with up to about one in five patients developing cancer. Because the abnormal immune response in SSc can damage a wide range of tissues, it’s been suspected that this damage may extend to DNA, thus giving rise to cancer-causing mutations. |
AuthorScleroderma Queensland Support Group Archives
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