Understanding Sjögren’s Diagnosis Criteria: A Comprehensive Guide

Diagnosing Sjögren’s Syndrome can often be a complex and challenging process. This is primarily because Sjögren’s manifests differently in each individual. Patients may present with a wide array of symptoms, and not everyone will test positive for all markers typically associated with the condition. Adding to the complexity, dryness, commonly considered a hallmark symptom, might not even be present at the onset, particularly in younger patients. In these cases, symptoms might lean more towards parotitis, specific serological findings, neurological and kidney-related issues, and general signs like fever or swollen lymph nodes.

Recognizing the diverse ways Sjögren’s can present is crucial for both patients and healthcare professionals. A thorough understanding of the various symptoms, as detailed by resources like the Sjögren’s Foundation, is a vital first step in considering Sjögren’s as a potential diagnosis. Ultimately, a Sjögren’s diagnosis isn’t solely based on a single test or symptom but rather on a comprehensive evaluation that combines clinical signs and patient-reported symptoms.

In 2016, a significant step towards standardizing the approach to Sjögren’s in research settings was taken with the establishment of the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) Consensus Sjögren’s Classification Criteria. It’s important to understand that these criteria were specifically designed for classifying patients for inclusion in clinical trials, not as a definitive diagnostic tool for everyday clinical practice. Therefore, diagnosing Sjögren’s in a clinic often remains nuanced, relying on a physician’s expertise to interpret symptoms alongside objective test results.

The ACR-EULAR 2016 Classification Criteria: Purpose and Application

The ACR-EULAR 2016 Classification Criteria serve a vital role in standardizing patient selection for Sjögren’s clinical trials. For any clinical trial to yield reliable and comparable results, patient groups need to be defined using strict, uniform criteria. These “Classification Criteria” are designed for this specific purpose and should not be misconstrued as rigid diagnostic rules for daily clinical management and treatment decisions.

However, despite their primary focus on research, these classification criteria offer valuable guidance for clinicians. They highlight key symptoms and objective tests that are strongly linked to Sjögren’s, providing a structured framework for considering the diagnosis. It’s also important to note that classification criteria are not static; they are regularly updated as new research and diagnostic tools become validated.

The 2016 ACR-EULAR criteria represent the most recent consensus on classifying Sjögren’s for research. These were developed through a collaborative effort involving international experts and the Sjögren’s Foundation, and subsequently endorsed by both the American College of Rheumatology and the European League Against Rheumatism. The criteria are structured around a three-step process:

1. Initial Symptom Assessment: The first step involves identifying patients who are suspected of having Sjögren’s. This suspicion can arise either from a positive finding in at least one domain of the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) or by answering affirmatively to at least one of the following symptom-based questions:

   • Have you experienced persistent, bothersome dry eyes every day for more than three months?
   • Do you frequently have a sensation of grit or sand in your eyes?
   • Do you use artificial tears or lubricating eye drops more than three times daily?
   • Have you had daily dry mouth for over three months?
   • Do you frequently drink liquids to help swallow dry food?

2. Exclusionary Conditions: The second step is to rule out other conditions that can mimic Sjögren’s symptoms. Patients are excluded if they have a history of any of the following:

   • Prior radiation therapy to the head and neck region
   • Active Hepatitis C infection (confirmed by PCR testing)
   • Acquired Immunodeficiency Syndrome (AIDS)
   • Sarcoidosis
   • Amyloidosis
   • Graft-versus-host disease
   • IgG4-related disease

3. Weighted Scoring System: Finally, a diagnosis based on the classification criteria requires a weighted sum of scores from five different items. A patient must achieve a total score of 4 or more to meet the 2016 Classification Criteria for Sjögren’s in a clinical trial setting.

Image: Table outlining the weighted scoring system of the 2016 ACR-EULAR Sjögren’s Classification Criteria.

It’s also important to consider medication use when applying these criteria. The guidelines advise that patients who are taking anticholinergic medications, which can cause dryness, should be assessed for objective signs of salivary gland and ocular dysfunction only after they have discontinued these medications for an adequate period. This ensures that dryness symptoms are genuinely related to Sjögren’s and not medication side effects.

Evolution and Context of Sjögren’s Classification Criteria

While the 2016 ACR-EULAR criteria are currently the standard for most clinical trials, it’s crucial to recognize the historical context of Sjögren’s diagnostic approaches. The previous iteration, the 2002 American-European Classification Criteria for Sjögren’s, has been widely used in numerous clinical studies that continue to inform current healthcare practices.

The 2002 criteria are considered to be broader in scope, placing a greater emphasis on oral and ocular symptoms compared to the more recent 2016 criteria. Understanding both sets of criteria provides a more comprehensive perspective on how Sjögren’s diagnosis and classification have evolved over time.

As research progresses, the field anticipates the validation of new diagnostic tools, such as salivary gland ultrasound, and the discovery of biomarkers that can further refine Sjögren’s diagnosis. These advancements promise to enhance diagnostic accuracy and potentially lead to even more precise classification criteria in the future, further improving both clinical practice and research in Sjögren’s Syndrome.

References:

1. Yokogawa N, Lieberman SM, Sherry DD, Vivino FB. Features of Childhood Sjögren’s Syndrome in Comparison to Adult Sjögren’s Syndrome: Considerations in Establishing Child-Specific Diagnostic Criteria. Clin Exp Rheumatol. 2016 Mar-Apr;34(2),343-51.
2. Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X, International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017 Jan;69(1),35-45.
3. Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X, International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Ann Rheum Dis. 2017 Jan;76(1)9-16.
4. Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, Gottenberg J-E, Bootsma H, Mariette X, Vitali C, EULAR Sjögren’s Task Force. Ann Rheum Dis. 2010 Jun;(6),1103-9.
5. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH, European Study Group on Classification Criteria for Sjögren’s Syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8.