'Promising treatments' for Sjögren’s syndrome may enter clinical use in 3-5 years
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It is likely that one or more “promising treatments” currently being investigated for Sjögren’s syndrome will come into use within the next 3 to 5 years, according to a presenter at the 2021 Congress of Clinical Rheumatology-West.
“If you go to clinicaltrials.gov, you will find that there are about 25 compounds under investigation, either biologics or small molecules for Sjögren’s,” Fredrick B. Vivino, MD, MS, of Penn Presbyterian Medical Center, in Philadelphia, to attendees at the hybrid meeting. “But among all the things that have recently been studied and are currently being studied, the most promising treatments are what I have listed here.”
Those treatments, all the subjects of studies published in either 2019 or 2020, include combination therapy with hydroxychloroquine and leflunomide, combination therapy with rituximab (Rituxan; Genentech, Biogen) and belimumab (Benlysta, GlaxoSmithKline), the anti-CD40 antibody iscalimab (Novartis) and the BAFF receptor inhibitor and B-cell depletion agent ianalumab (Novartis).
“These are the ones that have made it through phase 2 trials and have shown positive results,” Vivino said. “One would predict that one or more of these treatment strategies will likely come into use sometime within the next 3 to 5 years.”
However, the lack of any “remittive agents” is just one of many unmet needs for patients with Sjögren’s syndrome, according to Vivino. Still lingering out of reach are better therapies for treatment-refractory dry mouth and dry eyes, as well as alternative options for the “70% who don’t respond to the treatment algorithm” for corneal neuropathy, he noted. New clinical practice guidelines for treating several major organ systems, including neurologic involvement, as well as a strategy that would help prevent lymphomas, are also needed.
Still, progress continues. Just this year, the Sjögren’s Foundation published the first clinical practice guidelines for managing pulmonary disease in Sjögren’s syndrome in the journal Chest, Vivino noted. According to these new guidelines, it is now recommended that every patient, including those who are asymptomatic, be screened for pulmonary disease.
“It’s recommended we do a thorough history and physical, looking for clues for respiratory involvement, that we get a baseline chest X-ray for every patient and that we get baseline pulmonary function tests for every patient,” Vivino said.
Patients who demonstrate symptoms of respiratory involvement should undergo a high-resolution chest CT scan and, if there is reason to suspect pulmonary hypertension, an echocardiogram with doppler flow, according to the guidelines.
In addition, patients with early interstitial lung disease in Sjögren’s syndrome should be treated with steroids as the first line, followed by mycophenolate mofetil or azathioprine.
“That really works in the majority of cases, at least in our experience,” Vivino said. “If it doesn’t, then what they recommend is you take a step backward and reassess what you are treating. Make sure you are not treating an infection or malignancy or amyloid in the lungs, which can occur in Sjögren’s.”
If the conclusion remains inflammatory, the next step in the guidelines would be either rituximab or calcineurin inhibitors. If the consensus is that the issue is mainly a fibrotic process, the treatment of choice would be nintedanib (Ofev, Boehringer Ingelheim).
Vivino noted that although there are no cures for Sjögren’s syndromes, it can nonetheless be treated. He recommended that providers triage patient issues based on eye, mouth and systemic symptoms.
“Please remember that we are dealing with a systemic disease that causes a lot of problems that compromises quality of life,” Vivino said. “Even though we can’t cure it, we can certainly treat it. I’d like you to think of people in terms of the eyes, the mouth and the systemic manifestations, and if you take that approach, I think you will rarely miss anything that needs your attention. What you choose depends on severity, whether they have had complications, and on the internal organs involved. We now have some clinical practice guidelines to help us deal with some of these problems, and more guidelines, fortunately, are on the way.”
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Vivino FB. Sjogren’s Syndrome: Current Therapy and Future Directions. Presented at: Congress of Clinical Rheumatology-West annual symposium; September 18-21, 2021 (hybrid meeting).
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