2016 ACR/EULAR Classification Criteria for Primary Sjögren’s Syndrome

The 2016 ACR/EULAR Classification Criteria for Primary Sjögren’s Syndrome apply to any individual who meets the inclusion criteria, does not have any condition listed in the exclusion criteria, and has a score ≥4 when summing the weighted items below:

Inclusion Criteria 

Inclusion criteria are met if at least one of the following ocular or oral dryness symptoms is present:

• Daily, persistent, troublesome dry eyes for more than 3 months.

• Recurrent sensation of sand or gravel in the eyes.

• Use of tear substitutes more than 3 times a day.

• Daily feeling of dry mouth for more than 3 months.

• Frequent drinking of liquids to aid in swallowing dry food.

* Inclusion criteria are also met when there is a suspicion of Sjögren’s disease based on the ESSDAI questionnaire (at least one domain with a positive item).

Exclusion Criteria

A prior diagnosis of any of the following conditions excludes classification as primary Sjögren’s disease because of overlapping clinical features or interference with criteria tests:

• History of head and neck radiation treatment.

• Active Hepatitis C infection (with positive PCR).

• Acquired immunodeficiency syndrome (AIDS).

• Sarcoidosis.

• Amyloidosis.

• Graft-versus-host disease.

• IgG4-related disease.

* Patients who normally take anticholinergic medications should be evaluated for objective signs of salivary hypofunction and ocular dryness after a sufficient washout interval, so these components reflect true oral and ocular dryness.

Items

1. Histopathologic examination should be performed by a pathologist with expertise in the diagnosis of focal lymphocytic sialadenitis; focus score is the number of foci per 4 mm², assessed using the protocol described in Daniels et al. (2011).

2. Ocular staining score is described in Whitcher et al. (2010). The van Bijsterfeld score is described in van Bijsterveld (1969).

3. Unstimulated whole saliva flow is described in Navazesh & Kumar (2008).

• Provides an objective, standardized approach to classify patients as having primary Sjögren’s disease.
• Supports scientific communication and eligibility/enrollment for research and clinical trials.
• Although not designed as a bedside diagnostic tool, it may help clinicians document key objective features and structure the workup.

Use in patients undergoing diagnostic evaluation for suspected primary Sjögren’s disease who have at least one symptom of ocular or oral dryness.

• Developed through international consensus and generally considered an advancement over the 2002 American European Consensus Group (AECG) and 2012 American College of Rheumatology (ACR) criteria.
• Removed the need for salivary gland scintigraphy and parotid sialography, and incorporates modern ocular staining assessments.
• Demonstrated high test performance in the validation process (~96% sensitivity and ~95% specificity).
• Intended for classification, not diagnosis; meeting criteria supports classification but does not confirm disease on its own.
• Not validated for secondary Sjögren’s disease.
• Evaluate for alternative causes of sicca symptoms to avoid misclassification.

A patient may have Sjögren’s disease without meeting classification criteria; persistent clinical suspicion should prompt comprehensive evaluation and management based on clinical judgment and specialist expertise, regardless of the score.

• For patients with Sjögren’s disease, initiate treatment tailored to their manifestations and disease severity, such as:
  
  • Symptomatic care for dry eyes and/or mouth (e.g., artificial tears, salivary stimulation, oral and eye hygiene). 
  • Management of other sicca symptoms (e.g., saline spray for nasal dryness, moisturizers for dry skin).
  • Immunosuppressive therapy (e.g., corticosteroids, steroid-sparing agents, biologics) may be appropriate for moderate to severe disease and/or systemic involvement.
• Care often requires a multidisciplinary team (e.g., rheumatology, ophthalmology, dentistry/oral medicine).

• A score ≥4 indicates that a patient who meets inclusion criteria (and has no exclusion criteria) can be classified as having primary Sjögren’s disease.

• Results are not diagnostic; interpret alongside clinical findings and consider specialist input and additional testing as needed.