Scleroderma can be unpredictable, so I'd rather be informed and take action by Tomisa Starr | July 16, 2025

​I had a chest CT scan in April 2024, and then another one last March. I’m experiencing a bit of anxiety and uncertainty about the most recent scan.

There are some things about having a rare chronic illness like scleroderma that I just can’t control. This frustrates me because I like to feel like I’m in control of things. When I’m not, it makes me stressed and anxious.

People with scleroderma have an increased risk of cancer. My chest CT scan last year showed an 8-mm spiculated nodule in one of my lungs. My recent scan showed that the nodule had apparently shrunk to 7 mm. I didn’t know what having this lung nodule might mean, so I needed to know more. I was concerned that it might have a connection to cancer.
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Even though I know some things about how scleroderma affects me, I still don’t think I have all the answers. Since scleroderma is a complex disease involving multiple bodily systems, it affects everyone differently. Knowledge is power, because you never know when a seemingly small thing might lead to a bigger issue, like cancer, for example.

Study found differing survival rates for each subgroup by Steve Bryson, PhD | July 8, 2025

Scientists in China have identified four distinct subtypes of systemic sclerosis-associated interstitial lung disease (SSc-ILD), each characterized by different clinical characteristics, a new study found.

With these four subtypes identified, the team then analyzed outcomes for each subtype and found differing survival rates and factors associated with mortality, which may help better predict and improve patient outcomes, according to researchers.

The new study, “A noval identification of 4 systemic sclerosis – interstitial lung disease subgroups using principal component analysis-based cluster analysis,” was published in BMC Pulmonary Medicine.

Five key clinical and imaging variables identified
In SSc, also known as scleroderma, scar tissue builds up in the skin and internal organs. The lungs are particularly vulnerable, which can lead to interstitial lung disease, or ILD. This disorder is marked by damage to the tissue around the tiny air sacs in the lungs, or alveoli, making breathing difficult.

Lung function tests, clinical exams, and CT scans are established ways to assess SSc-ILD, but they often fail to capture the disease’s variability among patients, the researchers noted. Surgical lung biopsy, which is regarded as the gold standard for ILD classification, is rarely performed in SSc-ILD patients due to the associated risks of complications.

To learn more, a research team in China conducted a cluster analysis using a combination of clinical variables and imaging results to identify subgroups of SSc-ILD patients with similar clinical characteristics. This may allow clinicians to tailor treatments to each patient’s disease manifestations, according to the team.

The researchers retrospectively reviewed the medical records of 103 adults with SSc-ILD, most of whom were women (84%). Nearly all (97.1%) had skin manifestations and tested positive for antinuclear antibodies (96.1%), the self-reactive antibodies (autoantibodies) commonly found in SSc that react against components of the cell’s nucleus. CT scans revealed extensive lung involvement in most patients.

The team extracted five key clinical and imaging variables from patient records. Blood test variables were white blood cell count, the levels of the self-reactive antibody anti-SCL-70, and the C-reactive protein-to-albumin ratio, an inflammation-based marker for mortality. The imaging variable was ILD features based on CT scans. The fifth variable was the Warrick score, a standard measure of the severity of lung involvement, with scores of 15 or more indicating severe lung disease.

Therapy for skin conditions aims to ease inflammation, scarring in scleroderma by Margarida Maia, PhD | July 1, 2025

Galderma is launching a Phase 2 clinical trial to test nemolizumab, an antibody the company is developing to ease inflammation and scarring, known medically as fibrosis, in systemic sclerosis (SSc).

Recruitment is planned to begin in North America, South America, and Europe in the second half of this year, with trial completion anticipated in 2028, according to a company press release.

The goal, per Galderma, is to test the therapy’s safety and early efficacy in adults with SSc. Participants will be randomly assigned to receive either the antibody or a placebo.

The researchers also will look at pharmacokinetics, or how nemolizumab moves into, through, and out of the body, as well as pharmacodynamics — the treatment’s effects in the body.

The company is also launching a separate Phase 2 clinical trial of nemolizumab for chronic pruritus of unknown origin, or CPUO, a skin condition characterized by persistent itching lasting longer than six weeks. CPUO has no known cause and no approved treatments.
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“Investigating nemolizumab in two new trials … underscores our commitment to addressing skin conditions with high unmet needs,” said Baldo Scassellati Sforzolini, MD, PhD, Galderma’s global head of research and development. “These trials may help us better understand these complex diseases and offer hope for patients seeking relief from these severe and potentially life-threatening conditions.”

Findings could help researchers develop more effective treatments
by Margarida Maia, PhD | June 24, 2025

​Levels of certain types of immune cells are abnormally high in some people with systemic sclerosis (SSc), and this may be linked to serious kidney and lung complications — such as renal crisis and interstitial lung disease (ILD) — according to a study of blood cells from patients.

“Taken together, our single-cell analysis of patient samples show specific abnormalities in distinct subsets of immune [cells] are associated with different clinical symptoms of systemic sclerosis, particularly organ manifestations,” Hiroshi Shimagami, the study’s first author from the University of Osaka in Japan, said in a university press release.

By identifying and tracking these immune cells, doctors may be able to intervene early to prevent severe complications, the study suggests. These findings could also help researchers develop more effective treatments for SSc.
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The study, “Single-cell analysis reveals immune cell abnormalities underlying the clinical heterogeneity of patients with systemic sclerosis,” was published in Nature Communications. Five of its authors work at Chugai Pharmaceutical, which codeveloped Actemra (tocilizumab), an approved medication for systemic sclerosis-associated interstitial lung disease (SSc-ILD).

More people are aware of the disease today than when I was diagnosed
by Tomisa Starr | June 19, 2025

For many years before I was diagnosed with scleroderma, my skin was normal but I had joint pain. This was while I was still a child. During childhood, I may have had circulation problems, because my hands and feet were always cold. But I hadn’t yet experienced the numbness and color changes of Raynaud’s phenomenon. I felt short of breath when I had never experienced that before. It wasn’t that I had never really felt short of breath at all, but what I felt was more than I was used to.

I seemed to have some symptoms of scleroderma, even back then. This may have been what is now known as “pre-scleroderma,” but I don’t know for sure. Maybe, I will never know.
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When I was younger, people would sometimes ask me why I wore gloves on warm days or why my skin looked a little different from normal. I was once asked if my skin was burned. I would tell them that I have scleroderma. Almost always, the person would say, “Sclero … what?” That doesn’t happen as much now as it did before.

Researchers grouped patients by internal involvement, not skin symptoms
by Michela Luciano, PhD | June 3, 2025

​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.

Lung, kidney fibrosis common too, suggesting need for 'multimodal treatment by Andrea Lobo, PhD | May 20, 2025

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.

Data from 2 trials show benefits with approved treatment over 4 years
by Andrea Lobo, PhD | May 13, 2025

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.

Researchers say results could help guide diagnostic efforts, treatment
by Andrea Lobo, PhD | May 6, 2025

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.

Finding suggest factors may limit diagnostic access, influence exposures
by Andrea Lobo, PhD | April 29, 2025

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.