World Sjögren’s Day shines light on a disease that too often goes years without diagnosis

Despite being the second-most common rheumatic autoimmune disease in the United States, Sjögren’s syndrome remains under-recognized, with patients often waiting 3 years for a proper diagnosis, according to the Sjögren’s Foundation.

The disease impacts nearly 4 million Americans — nine out of 10 being women — and can affect nearly any person. However, it is most commonly diagnosed among adults aged in their late 40s.

Sjögren’s syndrome often goes unrecognized or misdiagnosed due to its resemblance to other medical conditions and the onset of different symptoms at different times. As a result, an estimated 2.5 million Americans are currently undiagnosed despite suffering from Sjögren’s symptoms.

July 23 marks World Sjögren’s Day, a campaign intended to raise awareness for the millions of people suffering from Sjögren’s disease. To overcome the complexities of this disease, the Sjögren’s Foundation encourages patients to use their voice to tell their story.

In support of World Sjögren’s Day, Healio recaps the latest news and research concerning the disease, including determining risk factors, updates on clinical trials and more.

‘Proof of concept’ established for FcRn receptor blocker nipocalimab in Sjogren’s disease

Patients with Sjögren’s disease treated with nipocalimab, a novel neonatal Fc receptor blocker, demonstrate significant improvement vs. those who receive placebo at week 24, according to data presented at the EULAR 2024 Congress.

“Approximately 4 million individuals worldwide have Sjögren’s disease,” Terence Rooney, MD, vice president of rheumatology and immunology disease area leader at Johnson & Johnson Innovative Medicine, told Healio. “It is nine times more common in women than men. There are currently no treatments that directly address the underlying causes of Sjögren’s disease. A clear need exists for patients living with Sjögren’s disease to have advanced therapies that target the underlying cause and systemic nature of the disease.” Read more.

‘Major advancement’: Interferon signature may be helpful biomarker in pediatric Sjögren’s

Most pediatric patients with Sjögren’s disease displayed an interferon signature in their blood and parotid gland tissue, which could help confirm diagnoses and shorten treatment delays, according to data presented at EULAR 2024 Congress.

“This can actually be used and introduced in clinical practice, not only in patients with Sjögren's syndrome but also in children, actually, as a new diagnostic test,” Dirk Foell, MD, chair of the EULAR pediatric rheumatology committee, said during a press conference at the meeting. “That’s a major advancement.” Read more.

Patients with prior sinusitis have greater rheumatic disease risk

Patients with prior sinusitis demonstrated an increased risk for many rheumatic diseases, including antiphospholipid syndrome, Sjögren’s disease and seronegative rheumatoid arthritis, according to data published in RMD Open.

The researchers noted that the risk was most pronounced in cases of sinusitis reported within 5 to 10 years before rheumatic disease onset. Read more.

Urticarial Vasculitis, Part 2: Walking the Balance Beam of Allergy and Rheumatology

This episode of the Healio Rheuminations podcast, hosted by Adam J. Brown, MD, digs into the pathophysiology of urticaria vasculitis from anti-C1q to the story of bradykinin. Listen here.

Trial of tivanisiran for dry eye associated with Sjögren’s fails to meet primary endpoint

A phase 3 clinical trial of tivanisiran failed to meet the primary endpoint in the treatment of dry eye disease associated with Sjögren’s syndrome, according to a press release from Sylentis.

The primary endpoint of the randomized, double-masked, placebo-controlled trial was the evaluation of tivanisiran’s efficacy, including signs and symptoms. Read more.

Topline VIVACITY, DAHLIAS data: Nipocalimab bests placebo for myasthenia gravis, Sjögren’s

Nipocalimab met the primary endpoint in a pivotal phase 3 trial in patients with generalized myasthenia gravis, as well as in a phase 2 study in adults with Sjögren’s disease, according to topline results released by Johnson & Johnson.

Compared with placebo, nipocalimab achieved statistically significant reductions in myasthenia gravis symptom scores at weeks 22 to 24 in the phase 3 VIVACITY trial, according to a release from Johnson & Johnson. In the phase 2 DAHLIAS trial, the drug also showed statistically significant reductions in a Sjögren’s disease severity scale, ClinESSDAI, at 24 weeks compared with placebo. The drug was well-tolerated across both studies. Read more.

NIH grants Oklahoma Medical Research Foundation $5.8 million for Sjögren’s research

The Oklahoma Medical Research Foundation has received a $5.8 million award from the NIH for research into the diagnosis and management of Sjögren’s disease, according to a press release.

OMRF researchers Darise Farris, PhD, Joel Guthridge, PhD, and Christopher Lessard, PhD, were awarded the 4-year grant through the Accelerating Medicines Partnership in Autoimmune and Immune-Mediated Diseases Program. The group aims to develop less-invasive tools for diagnosis and identify new therapeutic targets for Sjögren’s disease, which has no cure. Read more.

References:

Sjögren’s Fact Sheet. Sjögren’s Foundation. https://147789.fs1.hubspotusercontent-na1.net/hubfs/147789/2023%20World%20Sjogrens%20Fact%20Sheet.pdf. Accessed June 26, 2024.

World Sjögren’s Day. Sjögren’s Foundation. https://sjogrens.org/get-involved/spread-awareness/world-sjogrens-day. Accessed June 26, 2024.