Immunosuppressants may help treat scleroderma with heart involvement

Primary heart involvement is a common complication of systemic sclerosis by Margarida Maia, PhD | February 11, 2025

Immunosuppressive medications may help to reduce inflammation in the heart and the overall burden of primary heart involvement that is associated with systemic sclerosis (SSc), a retrospective study suggests.

Larger studies are needed to confirm these findings, its researchers stated.
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The study, “Immunosuppressive therapy to treat newly diagnosed primary heart involvement in patients with systemic sclerosis: An Italian cardiac magnetic resonance based study,” was published in Seminars in Arthritis and Rheumatism.

Most of the study’s 35 patients showed signs of heart muscle inflammation
Primary heart involvement is a common complication of SSc, an autoimmune disease of the connective tissue that holds organs and tissues in place (also known as systemic scleroderma). Poorer outcomes are possible in people whose heart is inflamed or scarred, and currently, no treatments exist for SSc with primary heart involvement.

Researchers in Italy assessed how well immunosuppressants can reduce heart inflammation in patients with primary heart involvement. To track changes in heart health before and after treatment, they used a detailed imaging technique called cardiac magnetic resonance or CMR.

Out of 684 SSc patients being followed at two centers in that country, 35 (5.1%) had confirmed primary heart involvement. Most of them (77.1%) were women, and the group’s median age was 59. More than one-third (34.3%) had diffuse systemic scleroderma. At the study’s start, many (74.3%) showed signs of heart muscle inflammation, such as edema (swelling) or a measure known as increased T2-mapping on CMR.

Patients were treated with various immunosuppressive medications for their disease’s primary heart involvement, including mycophenolate mofetil, rituximab, and azathioprine. Treatment lasted a median of 12 months. Follow-up CMR imaging, done after six to 16 months, revealed significant reductions in heart muscle inflammation.

T2-mapping, a key measure, improved from 53 to 51 milliseconds. Another measure, native T1-mapping (which detects damage to heart tissue), decreased from 1,050 to 1,039 milliseconds. The extracellular volume fraction, a marker of tissue scarring, also decreased from 34% to 33%.

In addition to imaging results, blood markers improved. NT-proBNP (which indicates heart strain), high-sensitive troponin T (heart injury), and C-reactive protein (inflammation) all decreased significantly. These changes suggest that treatment eased both heart inflammation and overall clinical burden.

“Immunosuppression is a therapeutic strategy which has the potentiality to treat newly diagnosed SSc [with primary heart involvement], by curbing signs of myocardial inflammation at CMR, and by significantly reducing cardiac enzymes, inflammatory markers and overall clinical burden,” the researchers wrote.

No treatment-related side effects were reported during follow-up.
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“Larger prospective randomized studies with longer follow-up are needed to confirm these preliminary data,” the scientists concluded, adding that their study’s findings also were limited by its small size.