Historical Review Traces Scientific Progress in Sjögren Disease

Much about Sjögren disease (SjD) remains under debate more than 90 years after Henrik Sjögren’s 1933 doctoral thesis sparked interest in the condition, according to a narrative and historical review published in the European Journal of Oral Sciences. The authors, from universities in Brazil, traced the history of SjD from its initial descriptions to more recent developments.¹

SjD is an autoimmune disorder that primarily affects the exocrine glands, specifically the salivary and lacrimal glands, causing dryness of the eyes and mouth. The disease is named for Sjögren, a Swedish ophthalmologist whose thesis focused on keratoconjunctivitis sicca.

Recognition as a Distinct Autoimmune Disease

Researchers added SjD was often overshadowed by other rheumatic diseases and struggled for recognition as a distinct autoimmune disease. Research in the 1960s and 1970s produced inconsistent nomenclature for its autoantibodies, which further complicated understanding of the disease.

The name of the disease has also been revised. In June 2025, an international task force published consensus recommendations in Nature Reviews Rheumatology stating that “Sjögren disease” should replace “Sjögren syndrome,” with SjD as the abbreviation. Researchers explain the previous name had proven unsatisfactory. Patients had perceived “syndrome” as indicating a vague collection of symptoms, which prompted the Sjögren’s Foundation to abandon the term.³

The recommendations were developed through a bibliometric analysis, a systematic literature review, and a Delphi consensus process, with equal involvement of professionals and patient representatives. Researchers also made 2 further recommendations:

• “Associated” should replace “secondary” when SjD occurs alongside a second systemic autoimmune disease that meets classification criteria.
• In clinical diagnosis, “Sjögren disease” is the preferred term without differentiating primary and associated forms. That differentiation is recommended for scientific studies, to define a homogeneous population.

According to the consensus, the name acknowledges the disorder’s distinct pathogenesis and aims to improve clarity in clinical practice and research.

Classification Criteria, Biopsy, and Ultrasound

Diagnostic criteria for SjD have significantly evolved, with more than 10 classification criteria proposed since 1965.¹ Diagnosis typically draws on the 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria. These incorporate anti-SSA (Ro) antibodies and objective measures such as Schirmer testing and salivary flow rates.²

According to the review, although Sjögren studied biopsies early on, standardized approaches to biopsy sites and histologic findings took considerable time to establish. More recently, salivary gland ultrasound has been introduced as a noninvasive diagnostic tool.¹

The authors noted that accurately assessing disease activity remains challenging because of the wide range of SjD manifestations. In addition to dryness, the disease can cause fatigue and pain and can affect organs such as the kidneys, gastrointestinal tract, and lungs.²

Sjögren Disease Treatment Pipeline

There is no FDA-approved disease-modifying therapy for SjD, and management is largely symptomatic. The status of 3 targeted therapies:

• Ianalumab (Novartis): In the phase 3 NEPTUNUS-1 (NCT05350072) and NEPTUNUS-2 (NCT05349214) trials, monthly ianalumab 300 mg produced a greater reduction in EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) scores at week 48 than placebo. A quarterly dosing arm in NEPTUNUS-2 did not significantly differ from placebo. The drug received FDA breakthrough therapy designation on January 16, 2026.
• Dazodalibep (Amgen): Met its week-48 ESSDAI primary end point in the phase 3 OASIZ 301 trial, which enrolled about 621 adults with moderate to severe systemic disease activity, according to topline results reported September 22, 2026. Numerical results have not been disclosed.
• Telitacicept (RemeGen): Approved for SjD by China’s National Medical Products Administration on June 8, 2026.

As a narrative and historical review, the article does not report new data.¹ The Sjögren’s Foundation estimates that about 2.5 million Americans with the disease are undiagnosed and that the average time to diagnosis is 3 years. No single test confirms SjD, and the disease symptoms can mimic or overlap with those of menopause, allergies, or other conditions.²

References

1. Cantali DU, de Oliveira FR, Machado DC, Bauer ME. Charting the course of Sjögren’s disease: a historical analysis of scientific progress. Eur J Oral Sci. Published online September 30, 2026. doi:10.1111/eos.70147
2. Joszt L. Pipeline advances in Sjögren disease bring targeted therapies closer. AJMC. October 2, 2026. Accessed October 13, 2026. https://www.ajmc.com/view/pipeline-advances-in-sj-gren-disease-bring-targeted-therapies-closer
3. Ramos-Casals M, Baer AN, Brito-Zerón MDP, et al; International Task Force on Nomenclature of Sjögren Disease. 2023 International Rome consensus for the nomenclature of Sjögren disease. Nat Rev Rheumatol. 2025;21(7):426-437. doi:10.1038/s41584-025-01268-z