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.
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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.
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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.
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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.
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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.
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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.

Certain autoantibodies tied to lungs and kidneys, study finds by Andrea Lobo, PhD | November 12, 2024

The specific self-reactive antibodies (autoantibodies) of people with systemic sclerosis (SSc) overlap syndrome — a condition in which SSc patients have clinical signs and autoantibodies related to other connective tissue diseases — are tied to organ damage and SSc prognosis, according to a single-center study in China.

Patients with anti-Scl-70 antibodies were more likely to have lung and kidney involvement, while anti-centromere antibodies (ACA) were tied to a lower risk of lung involvement, the study found.

“Rheumatologists may pay more attention on autoantibodies in the clinical practice, so as to provide evidence for further accurate disease assessment and treatment,” the researchers wrote.
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The study, “Autoantibodies in systemic sclerosis overlap syndrome and their correlation with organ damage and survival,” was published in Rheumatology.
SSc is an autoimmune disease characterized by the accumulation of scar tissue in the skin and potentially internal organs, including the heart, kidney, lungs, and digestive tract. SSc overlap syndrome patients have clinical features or autoantibodies related to connective tissue disorders such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) — the most common form of lupus — and Sjögren’s disease.

A columnist finds out about scleroderma from the 'National Enquirer' by Tomisa Starr | November 6, 2024

I’m 61 years old and live in a city over 100 miles away from a scleroderma care center. I’ve been diagnosed with the disease several times over the past 30 years, and it took me an equal amount of time to get referred to the Stanford University Scleroderma Center in California. I’m hoping to start going there for care next month.
My scleroderma symptoms began in childhood when I was about 8 years old. I had shortness of breath and lightheadedness, and I couldn’t keep up with the other kids running around on the playground. These problems might’ve gone unnoticed, but thankfully, a teacher told my mother I looked like I was going to pass out on the playground.
My pediatrician diagnosed me with a mild case of rheumatic fever, and I had to get blood tests a couple times a month for about two years. No one I knew had ever heard of scleroderma, not even my pediatrician.

Developer planning to launch Phase 2 trials in fibrotic diseases in 2025
by Patricia Inácio, PhD | October 29, 2024

CAL101, a therapy candidate for fibrosis (scarring) in scleroderma and related disorders, was found to be safe and well tolerated in a Phase 1 clinical trial, according to new data from the study.

Developed by Calluna Pharma, CAL101 is a first-in-class antibody that targets S100A4, a protein previously associated with SSc and idiopathic, or of unknown cause, pulmonary fibrosis.

Calluna expects to test the investigational therapy in Phase 2 trials of fibrotic and fibrotic-inflammatory-related disorders in 2025, the company said in a press release.
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“We are encouraged by the findings from the Phase 1 study. These results are an important step forward in the development of our lead asset, CAL101, particularly for fibrotic and fibro-inflammatory diseases where there remains a critical need for innovative therapeutic options,” said Jonas Hallén, MD, PhD, co-founder and chief medical officer of Calluna. “We are excited as we now move into the next phase of clinical development.”

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.