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Pulmonary hypertension may be scleroderma marker of prognosis

11/6/2025

 
Researchers grouped patients by internal involvement, not skin symptoms
by Michela Luciano, PhD | June 3, 2025
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​The onset of pulmonary hypertension (PH), or high blood pressure in the lungs, may be a critical factor in predicting disease progression and poorer survival in systemic sclerosis (SSc), a study from France suggests.

By grouping patients based on internal organ involvement, rather than relying on traditional classifications based on skin symptoms, the researchers found that the group that included patients who developed PH had significantly lower overall survival rates. The study, “Phenotypes and prognosis of systemic sclerosis: A cluster analysis,” was published in Respiratory Medicine and Research.

SSc, or scleroderma, is an autoimmune disease that causes hardening and scarring of the skin, along with the buildup of scar tissue in internal organs, particularly the lungs, heart, and kidneys, leading to a range of symptoms. Patients have been traditionally classified based on the extent of skin involvement, but this approach often fails to reflect how severely internal organs are affected or to predict how the disease will progress.
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Besides lung complications such as PH and interstitial lung disease (ILD) — a group of conditions that cause inflammation and scarring in the lungs — other poor prognostic factors in SSc include being male, kidney or heart involvement, and being older.

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Heart fibrosis may contribute to sudden death in systemic sclerosis

27/5/2025

 
Lung, kidney fibrosis common too, suggesting need for 'multimodal treatment by Andrea Lobo, PhD | May 20, 2025
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Most people with systemic sclerosis (SSc) have fibrosis, or scarring, that affects the heart muscle, a study in Australia shows.

In two-thirds of the cases, heart fibrosis had no other, or secondary, cause, helping to explain mortality and sudden cardiac death in these patients, according to the researchers. Lung and kidney fibrosis were also common in SSc.

Also, combinations of fibrosis, inflammation, and/or vascular disease in the heart, lungs, and kidneys were identified in 30% to 45% of SSc cases, “suggesting that multimodal treatment may be needed to prevent organ damage and improve outcomes in SSc,” the scientists wrote. The study, “Pathological contributors to organ damage and mortality in systemic sclerosis: a nationwide matched case-control study,” was published in Seminars in Arthritis and Rheumatism.
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SSc is an autoimmune disease marked by inflammation and fibrosis in the skin that may also occur in the internal organs, particularly the heart, lungs, and kidneys. In fact, internal organ involvement is a strong predictor of mortality with the condition, the scientists said, particularly due to cardiopulmonary and kidney disease.

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Ofev consistently slows lung function decline in SSc-ILD: Study

20/5/2025

 
Data from 2 trials show benefits with approved treatment over 4 years
by Andrea Lobo, PhD | May 13, 2025
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Lung function decline among people with systemic sclerosis-associated interstitial lung disease (SSc-ILD) was significantly slowed over the course of about four years of treatment with Ofev (nintedanib), according to a new analysis of trial data.

The analysis was based on changes in forced vital capacity, or FVC, a measure of lung function, throughout the Phase 3 SENSCIS trial (NCT02597933) and the open-label extension SENSCIS-ON (NCT03313180). It compared participants who were first given Ofev in the main study and those who started on a placebo and switched to Ofev in the extension study.

“The trajectories of decline in FVC in the SENSCIS and SENSCIS-ON trials illustrate the progressive nature of SSc-ILD in the population studied and support the efficacy of [Ofev] on slowing decline in lung function in these patients over the longer term,” the researchers wrote.
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The study, “Trajectories of forced vital capacity in patients with systemic sclerosis-associated interstitial lung disease,” was published in Arthritis Research & Therapy. Two of the study authors work at Boehringer Ingelheim, the company that markets Ofev, while other study authors have received funding from the company.

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Large study IDs risks for primary heart involvement in scleroderma

9/5/2025

 
Researchers say results could help guide diagnostic efforts, treatment
by Andrea Lobo, PhD | May 6, 2025
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Wasting of the skeletal muscle, older age, being male, and having swollen joints are among the risk factors for primary heart involvement associated with systemic sclerosis (SSc), according to a study using a worldwide database.

Particularly, intestinal symptoms, widened blood vessels underneath the skin, called telangiectasia, and older age were linked with a greater risk of a new onset of SSc-primary heart involvement (SSc-pHI), while swollen joints increase the risk for heart disease progression.

“Our results could help to stratify patients with SSc according to the risk of development or progression of SSc‐pHI to guide diagnostic efforts and treatment initiation,” the study’s researchers wrote. The study, “Evaluation of Systemic Sclerosis Primary Heart Involvement and Chronic Heart Failure in the European Scleroderma Trials and Research Cohort,” was published in the Journal of the American Heart Association.

In systemic sclerosis, or scleroderma, inflammation and scar tissue accumulation (fibrosis) occur in the skin and internal organs, including the heart and lungs. Primary heart involvement, when cardiac abnormalities are predominantly attributed to SSc, is one of the leading causes of death in SSc.

​It isn’t possible to “predict whether individual patients will develop clinically relevant SSc-pHI, whether it will be progressive, and how it may respond to immunomodulatory or antifibrotic therapies,” wrote an international team of researchers who analyzed data from the EUSTAR (European Scleroderma Trials and Research) database to learn more about risk factors and outcomes in SSc-pHI. A total of 5,741 patients were included in the analysis.

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Age, sex, socioeconomics tied to localized scleroderma risk: Study

9/5/2025

 
Finding suggest factors may limit diagnostic access, influence exposures
by Andrea Lobo, PhD | April 29, 2025
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People older than 65 and women are more likely to develop localized scleroderma, a study in the U.S. shows.The risk is lower in African Americans.
A higher risk of the disease was also seen in people who are unemployed, whereas those with less educational achievements, those whoo have less than a high school education, and lower income were at a reduced risk.

“Ultimately, our findings advocate for clinicians to integrate demographic factors such as gender, race, and socioeconomic status into their approach to [localized scleroderma] diagnosis and management,” the researchers wrote. The study, “Socioeconomic factors associated with the development of localized scleroderma: a cross-sectional study,” was published as a letter to the editor in the Archives of Dermatological Research.

Scleroderma is an autoimmune disease that affects the skin and connective tissue, which supports and holds organs together. The hallmark symptom is the accumulation of thick and hardened skin caused by excessive production of collagen, the main component of scar tissue. Localized scleroderma most often affects the skin and muscles, and rarely affects the internal organs.
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Researchers at the Rutgers Robert Wood Johnson Medical School in New Brunswick analyzed the electronic health records of people enrolled in the All of Us database to better understand the patterns of a localized scleroderma diagnosis in underrepresented patient populations.

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Exosome-based therapy eases skin scarring in scleroderma mice: Study

29/4/2025

 
Findings could lead to new scleroderma treatment, researchers say by Steve Bryson, PhD | April 15, 2025
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​Exosomes, tiny sacs containing biomolecules secreted from stem cells, thinned skin scarring in a mouse model of scleroderma, as well as skin samples from patients, a study reported.
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These findings support “the use of exosomes as a clinical treatment for [scleroderma] skin fibrosis,” the researchers said in the study, “Exosomes carrying adipose mesenchymal stem cells function alleviate scleroderma skin fibrosis by inhibiting the TGF-β1/Smad3 axis,” which was published in Scientific Reports.

Scleroderma is an autoimmune disorder marked by the abnormal accumulation of collagen, the main protein component of scar tissue, in the skin and various organs. It’s called localized scleroderma when it affects just the skin and/or underlying muscle tissue, and systemic scleroderma when organs, including the heart, lungs, stomach, and kidneys, are also involved.
Nearly all scleroderma patients have skin involvement, and the severity of skin scarring, or fibrosis, is typically associated with damage to the vital organs and mortality.

Mesenchymal stem cells, or MSCs, are a type of stem cell found in various tissues, including the bone marrow, adipose (fat) tissue, and the umbilical cord. Due to their anti-fibrotic and anti-inflammatory properties, MSCs have been considered a promising treatment for scleroderma.
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MSCs work by releasing signaling proteins and/or exosomes, tiny membrane-bound sacs that carry proteins and other molecules important for cellular communication. Because exosomes are cell-free, they may circumvent the adverse effects associated with MSC therapy, including immune rejection.

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ALLO-329 gets FDA fast track designation for diffuse scleroderma

15/4/2025

 
Car T-cell therapy aims for off-shelf cell supply by Marisa Wexler, MS | April 8, 2025
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The U.S. Food and Drug Administration (FDA) has granted fast track designation to the cell therapy ALLO-329 to treat active refractory (resistant to treatment) diffuse scleroderma, also known as diffuse systemic sclerosis (SSc).

The FDA also gave ALLO-329 fast track designation as a potential treatment for two other inflammatory diseases: myositis, which is characterized by muscle inflammation, and lupus, which is marked by antibodies that attack the body’s healthy tissues.

Fast track designation is designed to speed the development of new treatments that have the potential to address unmet medical needs for serious or life-threatening diseases. With these designations, ALLO-329’s developer Allogene Therapeutics will get access to benefits including more frequent interactions with the FDA throughout the drug development process.

“Receiving these designations for ALLO-329 underscores the versatility and transformative promise of this next-generation allogeneic CAR T investigational product in redefining the autoimmune treatment landscape,” said Zachary Roberts, MD, PhD, executive vice president of research and development and chief medical officer of Allogene, said in a company press release.
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ALLO-329 is a CAR T-cell therapy. T-cells are immune cells that are able to kill other types of cells. CAR T-cell treatments involve engineering T-cells to equip them with a chimeric antigen receptor (CAR), a molecular weapon that directs a T-cell to attack other cells bearing specific molecular markers. ALLO-329 uses a CAR that targets CD19 and CD70, two proteins that are present, respectively, on the surface of B-cells and T-cells, both of which are immune cells involved in the development of many autoimmune diseases.


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Finding the ‘Goldilocks zone’ to manage scleroderma, Raynaud’s

15/4/2025

 
I like to go for walks when it's not too hot or cold by Tomisa Starr | April 2, 2025
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For those of us with scleroderma, it’s important to stay warm, because changes in body temperature, especially when exposed to the cold, can cause blood vessels to constrict.

I like to go for walks to get exercise and maintain my lung function. My exercise “Goldilocks zone” — my favorite time of the year to go for walks — is when it’s not too cold or hot outside. Where I live in California, that occurs from late fall to spring, when the daily high temperatures are in the mid 50s to 60s. Exercise makes my blood flow, which helps to keep me warm.
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Raynaud’s phenomenon, a condition where the fingers and toes feel numb, prickly, and frigid in response to cold temperatures or stress, is common among people with scleroderma. The constriction of blood vessels can cause color changes in the fingers. My fingers look purple, yellow, and red when Raynaud’s affects me.

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Ofev effectiveness, safety seen over 3-year SENSCIS extension trial

2/4/2025

 
Sustained slowing of lung function decline shown for SSc-ILD patients
by Andrea Lobo, PhD | March 25, 2025
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Treatment with Ofev (nintedanib) for up to four years was associated with a sustained slowing of lung function decline in people with systemic sclerosis (SSc) associated with interstitial lung disease (SSc-ILD).

That’s according to new results from the open-label study SENSCIS-ON (NCT03313180), a three-year extension of the SENSCIS Phase 3 trial that formed the basis for the therapy’s regulatory approvals. In the long-term extension, Ofev also had a safety profile consistent with previous studies, the new data show.
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“These findings suggest that [Ofev] can be used over the long term to slow the progression of SSc-ILD and so improve patient outcomes,” the researchers wrote.
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Their study, “Continued nintedanib in patients with systemic sclerosis-associated interstitial lung disease: 3-year data from SENSCIS-ON,” was published as a brief report in the journal RMD Open Rheumatic & Musculoskeletal Disorders.

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Phase 1 trial of CAR T-cell therapy likely to open soon to SSc patients

21/3/2025

 
Safety study testing ADI-001 in adults with various autoimmune diseases
by Margarida Maia, PhD | March 18, 2025
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Adicet Bio expects adults with systemic sclerosis (SSc) soon to start enrolling in its ongoing Phase 1 clinical trial of ADI-001, an experimental CAR T-cell therapy that the company is developing for a number of autoimmune diseases.

The company had expected sites to open to SSc patients before the end of last year, and now anticipates recruitment to be underway by the close of June. The open-label Phase 1 clinical trial (NCT06375993) currently is continuing to enroll adults with lupus nephritis, a common complication of the autoimmune disease lupus.

Depending on the speed with which people with SSc are enrolled, preliminary data on treated patients might be available toward the close of 2025.

ADI-001 targets B-cells, aiming to prevent damaging immune system attacks
ADI-001 recently received fast track status in the U.S. for treating adults with SSc, which works to speed the development and review of therapies for serious diseases with an unmet need.

This designation “highlights the significant unmet need for innovative, off-the-shelf therapies to treat autoimmune diseases,” Chen Schor, president and CEO of Adicet Bio, said in a company press release reporting on last year’s financial results and recent progress.

Systemic sclerosis, also called systemic scleroderma, is a multisystem autoimmune disease marked by the accumulation of scar tissue in the skin and organs that can include the heart, kidneys, lungs, and digestive tract.

Autoimmune diseases like SSc occur when the immune system produces antibodies that mistakenly react against healthy tissues in the body, guiding a damaging immune attack. Antibodies, including self-reactive ones, are produced by immune B-cells.

ADI-001 involves collecting a patient’s immune T-cells and modifying them in the lab before returning them via an infusion. T-cells are modified such that they produce a chimeric antigen receptor, or CAR, that binds to CD20, a protein found on the surface of B-cells.

Most of the T-cells present in ADI-001 are of a subtype called gamma delta T-cells, which naturally target various tissues, according to the company. Once CAR T-cells bind to CD20, they are activated to destroy B-cells, as reported in early clinical data from people with B-cell cancers, another indication for which the therapy is being developed.
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By killing B-cells, ADI-001 is expected to reduce the production of self-reactive antibodies, easing symptoms in people with autoimmune diseases.

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