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Advancements in Sjögren’s Disease

CE / CME

Making Progress in Sjögren’s: Key Considerations for Improving Recognition, Disease Management, and the Potential of Emerging Therapies

Physician Assistants/Physician Associates: 1.00 AAPA Category 1 CME credit

Nurse Practitioners: 1.00 Nursing contact hours, includes 1.00 hour of pharmacotherapy credit

Released: November 21, 2024

Expiration: November 20, 2025

R. Hal Scofield
R. Hal Scofield, MD
CME/CE Information

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Sjögren’s Syndrome Pathophysiology

Patients with Sjögren’s syndrome experience both genetic and environmental influences, though the specific environmental triggers remain largely unidentified. It is clear that the disease progresses through a stage of benign autoimmunity, where autoantibodies—such as anti‑Ro, anti‑La, and ANAs—can appear many years before clinical disease. Studies led by Roland Jonsson in Scandinavian countries demonstrated this, using blood samples from individuals who later developed Sjögren’s. These studies revealed the presence of autoantibodies long before the disease became clinically apparent.8-10

Patients with progressive disease show not only the presence of antibodies but also pathogenic changes, such as lymphocytic infiltration of the salivary glands, which eventually leads to clinical symptoms like severe dryness and extraglandular manifestations. The exact role of genetics on each stage of this progression, as well as environmental factors, is still not fully understood.8-10

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Potential Disease Triggers

The pathogenesis of Sjögren’s Syndrome is clearly driven by immune system involvement, particularly through infiltration of T-cells and B-cells into the salivary glands. This infiltration is a hallmark of the disease and can be studied through minor salivary gland or parotid biopsies.11

Patients with Sjögren’s syndrome exhibit autoreactive T-cells and B-cells within the salivary glands, where ectopic germinal centers form. In these centers, B-cells are “educated” by follicular T-cells. This interaction is mediated by elevated cytokines, including interferons.

Disrupting the interaction between T-cells and B-cells is a key strategy for development of disease-altering drugs for Sjögren’s syndrome. Targeting the interaction between B-cells and T-cells, particularly the cytokines and surface molecules that facilitate their communication, represents a promising target for future therapies. In this context, CD40 and CD40 ligand are key players in the interaction between T-cells and B-cells and should be considered important therapeutic targets.

B-cell–activating factor (BAFF) is another potential therapeutic target, as is the Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling pathway, which is involved in the expression of interferons.11

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