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4 Articles in this Series
Introduction
Diagnosing and Treating Sjogren's Syndrome More than Meets the Eye
Retaining Mobility May Lead to Pain Reduction
Treatment Uncertainty Remains for Asymptomatic Hyperuricemia
Vitamin D Supplementation May Improve Chronic Widespread Pain

Diagnosing and Treating Sjogren's Syndrome More than Meets the Eye

With Frederick Vivino, MD, and Bao Nguyen, DO, PhD

Sjogren's syndrome, a chronic, systemic autoimmune rheumatic disease marked by lymphocytic infiltration of exocrine glands and other organs in association with the production of various autoantibodies in the blood, typically develops insidiously over a period of months or even years. In addition to dry eyes (eg, a lack of tears), the most common symptoms include dry mouth, fatigue, musculoskeletal pain, and swelling of the major salivary glands.1

Diagnosis and management are not always straightforward, according to Frederick Vivino, MD, FACR, director of the Penn Sjogren's Syndrome Center and professor of clinical medicine at the Perelman School of Medicine at the University of Pennsylvania. He focused on the challenges of diagnosing this complex autoimmune disease in a "Meet the Professor" discussion at the American College of Rheumatology/Association of Rheumatology Health Professionals (ACR/ARHP) 2017 Annual Meeting.1,2

Not All Cases of Sjogren’s Syndrome Are Obvious

“About 80% of patients with Sjogren's syndrome have sicca, a dryness of the exocrine glands, including eyes and mouth, as well as other manifestations, such as lupus or rheumatoid arthritis,” said Dr. Vivino.

"The other 20% of cases will appear with atypical presentations," he said, with sicca symptoms less apparent or even with little to no evidence of dry eye or dry mouth.1

"With some individuals who complain most bitterly about sicca, it turns out to be some other condition," Dr. Vivino said. He cautioned clinicians to be on the lookout for medication-related dry mouth that has relation to Sjogren's syndrome, "In fact, more than 500 medications may lead to sicca symptoms."

The differential diagnosis of Sjogren’s syndrome necessitates ruling out a host of similar presentations,  such as mouth breathing, multiple sclerosis, fibromyalgia, hepatitis C, and many more conditions.1

Diagnostic Criteria are Extensive but Not Consistent

Twelve different classification and/or diagnostic criteria have been published between 1965 and 2017, said Dr. Vivino, "However, they all varied somewhat in the definition of what Sjogren's is, which tests are recommended, and offer difference diagnostic algorithms."

Then, in 2016, the ACR-EULAR criteria were issued.3 Even so, many clinicians are still using the 2002 criteria.1,4  These may be applied to any person with at least one symptom of ocular or oral dryness as defined by questions about frequency and intensity.5

As simple as it may sound, a complete history and physical are crucial, Dr. Vivino said, “All the classification criteria developed over the last 15 years may be useful in providing a framework for diagnostic testing.1  In addition, imaging may provide additional information about the involvement of major salivary glands.

“While extra-glandular manifestations typically present later in the disease onset, sometimes these symptoms are present at the beginning; therefore, it is important to remain vigilant in that any organ may be affected, especially the musculoskeletal system, gastrointestinal tract, lungs, and circulatory system,” said Dr. Vivino.

Another key diagnostic challenge arises in the complication of non-Hodgkin's B-cell lymphoma as a possible major complication, he said, typically occurring in 5 to 7% and usually within 10 years of diagnosis.6

Best Approach to Treat Remains Palliative

There is no cure or remittive agent is available.1 As such, treatment goals remain as follows:

  • Palliation of symptoms
  • Prevention of complications
  • Management of extra-glandular symptoms
  • Consideration of patients for immunosuppressive therapy

In 2016, the Sjogren's Syndrome Foundation published what may be considered the first US-based clinical practice guidelines for the condition.5

Among the recommendations:

  • Patients with xerostomia should be given fluoride for caries prophylaxis.
  • Dry eye treatment depends on assessment to evaluate severity level and the contribution of various factors.
  • Disease-modifying antirheumatic drugs can help inflammatory musculoskeletal pain.

Is There a Role for Biologics as a Treatment?

Recent studies about biological therapies for Sjorgren's have raised considerable interest.6 While the guidelines indicate that these products should not be used to treat sicca symptoms in those with primary disease, rituximab (Rituxan) may be considered in certain cases for keratoconjunctivitis sicca, xerostomia, and systemic symptoms.1,5

Physicians should also be aware of the potential adverse effects that come with use of rituximab, including infections, bowel obstruction, and other issues.

Clinical Perspective on More Effective Management of Sjogren's 

Diagnosing Sjogren's is often difficult and very challenging, said Bao Nguyen, DO, PhD, a rheumatologist from Houston, Texas who attended the session.

“To choose the best treatment, it is critical to find the most effective medicine but with the fewest side effects,” he told Practical Pain Management. "In managing these patients, it is particularly true to say that nothing is free," he says.

The newest developments gained from the session, Dr. Nguyen said, are the recommendations to consider Sjogren's syndrome even when dry eye is not the primary complaint, and use of rituximab as a viable treatment.

Dr. Vivino reports an association with Biogen. Dr. Nguyen has no disclosures.

Sources:

  1. Vivino F. Meet the professor—Managing Sjogren’s Syndrome. Presented at: The American College of Rheumatology 2017 Annual Meeting, November 3-8, 2017 in San Diego, California.
  2. Vivino, F. Sjogren's syndrome: Clinical aspects. Clin Immunol. 2017;182:48-54.
  3.  Vitali C, Bombardieri S, Jonsson R, et al, for the European Study Group on Classification Criteria for Sjögren’s Syndrome. Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61:554-558.
  4. Shiboski  CH, Shiboski  SC, Seror  R, et al, and the International Sjogren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjogren’s Syndrome — A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts,  Arthritis Rheumatol . 2017;69(1):35-45.
  5. Vivino, FB et al. New Treatment Guidelines for Sjogren's Disease. Rheum Dis Clin N Am. 2016;42(3) 531-551.
  6. Carsons SE, Vivino FB2, Parke A, et al. Treatment guidelines for rheumatologic manifestations of Sjögren's syndrome: Use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain. Arthritis Care Res.2017;69(4):517-527. 
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