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.