Janet Pope,<\/b> <\/b>MD<\/b>, told attendees. Image: Rob Volansky | Healio<\/i>
Systemic sclerosis (SSc) can impact multiple organ systems, most commonly the lungs and skin, according to Pope.<\/p>\n
\u201cThe damage is not reversible, but damage increases the risk of further damage,\u201d she said, noting that rheumatologists treating SSc can take their cues from other autoimmune diseases. \u201cWe are kind of working with a lupus paradigm.\u201d<\/p>\n
Because patients with SSc are most likely to die from lung complications, understanding the treatment landscape for pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) is critical to preventing fatalities. <\/p>\n
While FDA approvals are few and far between in SSc, sotatercept-csrk (Winrevair, Merck) has been approved for patients with PAH. \u201cThis can improve survival,\u201d Pope said. <\/p>\n
But one drug is unlikely to minimize damage or prevent mortality. Combination therapy with a phosphodiesterase type 5 (PDE5) inhibitor plus an endothelial receptor medication can be effective for PAH in SSc. \u201cThen add a third therapy such as a [prostaglandin] receptor agonist,\u201d Pope said. <\/p>\n
A few options are available for patients with ILD in SSc, according to Pope. \u201cThe PDE4 selective inhibitor nerandomilast added to nintedanib or on its own can prolong survival in progressive pulmonary fibrosis, including the subset with SSc-ILD,\u201d she told Healio. \u201cI might also add it to nintedanib.\u201d<\/p>\n
Rituximab (Rituxan, Genentech) or tocilizumab (Actemra, Genetech) can be added to mycophenolate mofetil (MMF) for ILD in SSc, as well, according to Pope.<\/p>\n
For skin fibrosis, methotrexate and rituximab may be used in the first line. \u201cWe want to treat early diffuse disease,\u201d Pope said. <\/p>\n
Hematopoietic stem cell transplantation (HSCT) has gained attention as a treatment of SSc, particularly in patients with skin fibrosis. \u201cIs it a cure?\u201d Pope said. \u201cNo. There is a chance of failure. But if I have patients with aggressive onset and early diffuse disease, with a high lung score, a high skin score, why not just give them everything?\u201d<\/p>\n
Pope told attendees that HSCT is not likely to fix all of the manifestations of SSc.<\/p>\n
\u201cI don\u2019t think a single therapy will be a home run for my patients,\u201d Pope said. \u201cWe need an orchestra of therapies working together.\u201d<\/p>\n
The success of chimeric antigen receptor T-cell replacement in lupus has spawned significant interest in cellular therapies to treat other autoimmune and rheumatic diseases. <\/p>\n
Pope noted that bispecific T-cell engager (BiTE) therapy may have utility in SSc.<\/p>\n
\u201cI think of it as a deeper B-cell depletion than rituximab,\u201d she said. \u201cIt is probably not as deep as CAR-T cell replacement, but it is easier to administer at less cost.\u201d <\/p>\n
Regarding CAR T cells, Pope noted a recent search of ClinicalTrials.gov showing close to 30 trials involving more than 300 patients currently under way. However, there does not appear to be a clear pathway to approval in a specific indication or for a specific patient population.<\/p>\n
\u201cWe need people to give us guidance on what should happen,\u201d she said. \u201cIf there is no pathway to approval, you have to wonder why this is being done. I wonder if we are putting the CAR T before the horse in scleroderma.\u201d <\/p>\n
The other issue pertains to cure. \u201cAre these people looking like they are cured at the end of these trials?\u201d Pope said. \u201cNo. we need more data. Monotherapy is not going to be the home run in systemic sclerosis.\u201d<\/p>\n
For more information:<\/h2>\n Janet E. Pope, MD, MPH, <\/b>FRCPC,<\/b> can be reached at Janet.Pope@sjhc.london.on.ca.<\/p>\n\n
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Published by:<\/span>\n
Sources\/Disclosures<\/h3>\n Source: <\/h2>\n Pope J. Novel therapies in systemic sclerosis. Presented at: Congress of Clinical Rheumatology East; April 30-May 3, 2026; Destin, Florida (hybrid meeting).<\/p>\n
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