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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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Introduction

In this module, Dr Hal Scofield discusses essential strategies for improving the diagnosis and management of Sjögren’s syndrome, and explores emerging therapies in later-stage development. 

The key points discussed in this module are illustrated with thumbnails from the accompanying downloadable PowerPoint slidesets, which can be downloaded here or by clicking any of the slide thumbnails in the module alongside the expert commentary.

Clinical Care Options plans to measure the educational impact of this activity. Some questions will be asked twice: once at the beginning of the activity and then once again after the discussion that informs the best choice. Your responses will be aggregated for analysis, and your specific responses will not be shared.

Before continuing with this educational activity, please take a moment to answer the following questions.

Which are examples of physiologic pathways that are targeted by agents in phase III studies for Sjögren’s syndrome?

A patient is complaining of dry eye and dry mouth, with persistently swollen salivary glands. What test or tests would provide a definitive diagnosis of Sjögren’s syndrome?

In phase II clinical trials, dazodalibep showed improvement in…

In phase II clinical trials, ianalumab showed improvement in…

Sjögren’s Syndrome: A Model Complex Autoimmune Disorder

Sjögren’s syndrome, also known as Sjögren’s disease, is an autoimmune condition in which the immune system primarily targets the exocrine glands, including the salivary and lacrimal glands. As a result, affected individuals commonly experience severe dry eyes and dry mouth. This disorder is relatively prevalent, potentially impacting over one million individuals in the United States, making it one of the more common autoimmune rheumatic diseases, second only to rheumatoid arthritis in terms of frequency.1

Sjögren’s syndrome can manifest in 2 forms: primary Sjögren’s syndrome, where it is the sole diagnosis, or secondary Sjögren’s syndrome, where it coexists with other autoimmune or rheumatic diseases such as systemic lupus erythematosus, rheumatoid arthritis, scleroderma, or primary biliary cirrhosis. However, some experts argue that it should not be classified “secondary” in these cases, but rather as a concurrent or associated disease.2

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Sjögren’s Syndrome Epidemiology

Sjögren’s syndrome typically presents in late adulthood and predominately affects women, with a female-to-male ratio of at least 10:1, possibly as high as 15:1. Many patients have a family history of autoimmune diseases, although the genetic basis of Sjögren’s syndrome is less well studied compared to other autoimmune conditions like rheumatoid arthritis or lupus.1,2

Sjögren’s syndrome is often misdiagnosed or underdiagnosed. Many patients present with a positive antinuclear antibody (ANA) test, leading to an initial diagnosis of lupus. Similarly, the presence of rheumatoid factor (RF) in patients with Sjögren’s may result in misdiagnosis of rheumatoid arthritis. On average, the time from onset of symptoms to a confirmed diagnosis is approximately 3 years.3

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Disease Burden

Patients with Sjögren’s syndrome can be categorized into 2 main groups: those with glandular disease and those with extraglandular disease. 

Patients with extraglandular disease may have involvement of multiple systems, including the articular, musculoskeletal, neurologic, and renal systems. Laboratory abnormalities such as hypergammaglobulinemia and complement deficiencies are also often observed. These patients tend to have a higher Sjögren’s Disease Activity Index (SDAI) and are considered to have high disease activity, though their symptom burden may vary.

On the other hand, patients with glandular disease, while primarily affected by severe sicca symptoms (dryness of the mouth and eyes), may also experience a significant symptom burden. Symptoms such as fatigue, insomnia and pain are common and can severely impact quality of life. Despite the absence of extraglandular manifestations, these patients may also report a high symptom burden. 

When surveyed, both patients with glandular disease and extraglandular disease consistently identify fatigue as the most burdensome aspect of their condition.4

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Risk Factors for Mortality

Patients with Sjögren’s syndrome who have high disease activity face several important mortality risk factors. A key concern is the increased lifetime risk of lymphoma, which ranges from 5% to 10% in these patients. This risk is much higher compared to other autoimmune diseases such as rheumatoid arthritis, which also carry an increased risk of developing lymphoma, but to a lesser extent than Sjögren’s.5

In addition to lymphoma, patients with high disease activity are at increased risk of cardiovascular disease and related events, as well as respiratory disease, which can contribute to both mortality and morbidity.6

Lastly, patients with high European Sjögren’s Syndrome Disease Activity Index (ESSDAI) scores over a long time have a small but significantly increased risk of mortality.6

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Risk Factors for Lymphoma in Sjögren’s Syndrome

There are several ways to assess a patient’s risk for lymphoma, with various predictive scoring systems incorporating multiple clinical features. Some of these key features include salivary gland enlargement, which is a common manifestation, as well as lymphadenopathy, which may involve cervical, axillary, or inguinal lymph nodes. Another common comorbidity with Sjögren’s is Raynaud’s phenomenon, which is risk factor for lymphoma. In addition, the presence of anti‑Ro and anti‑La antibodies, monoclonal gammopathy, and decreased complement levels are all associated with an increased risk of lymphoma.7

As shown in the graph, the number of these risk factors correlates with progressively higher risk of developing lymphoma, with patients possessing multiple risk factors being at higher risk compared to those with fewer. Remarkably, in one study, 100% of patients with 7 risk factors developed lymphoma, though the sample size was likely small. Nonetheless, the data emphasize the substantial risk for those with a high burden of predictive factors.7

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