ACR/EULAR classification criteria for primary Sjögren's syndrome

Item	Weight/score
Labial salivary gland with focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm²*	3
Anti-Ro/SSA positive	3
Ocular staining score ≥5 (or van Bijsterveld score ≥4) in at least one eye^¶^Δ	1
Schirmer test ≤5 mm/5 minutes in at least one eye^¶	1
Unstimulated whole saliva flow rate ≤0.1 mL/minute^¶^◊	1

The classification of primary SS applies to any individual who meets the inclusion criteria^§, does not have any of the conditions listed as exclusion criteria^¥, and has a score ≥4 when the weights from the five criteria items are summed.

ACR: American College of Rheumatology; EULAR: European League Against Rheumatism; SS: Sjögren's syndrome; PCR: polymerase chain reaction; IgG4: immunoglobulin G4.
* Labial salivary gland with focal lymphocytic sialadenitis and focus score ≥1 foci/4 mm². The histopathologic examination should be performed by a pathologist with expertise in the diagnosis of focal lymphocytic sialadenitis and focus score count, following a protocol described by Daniels, et al^[1].
¶ Patients who are normally taking anticholinergic drugs should be evaluated for objective signs of salivary hypofunction and ocular dryness after a sufficient interval without these medications in order for these components to be a valid measure of oral and ocular dryness.
Δ Ocular staining score described by Whitcher, et al^[2]; van Bijsterveld score described by van Bijsterveld^[3].
◊ Unstimulated whole saliva flow rate measurement described by Navazesh and Kumar^[4].
§ Inclusion criteria: These criteria are applicable to any patient with at least one symptom of ocular or oral dryness, defined as a positive response to at least one of the following questions: (1) Have you had daily, persistent, troublesome dry eyes for more than three months?; (2) Do you have a recurrent sensation of sand or gravel in the eyes?; (3) Do you use tear substitutes more than three times a day?; (4) Have you had a daily feeling of dry mouth for more than three months?; (5) Do you frequently drink liquids to aid in swallowing dry food? or in whom there is suspicion of SS from the EULAR SS Disease Activity Index questionnaire (at least one domain with positive item). Refer to UpToDate topic on the management and prognosis of Sjögren's syndrome.
¥ Prior diagnosis of any of the following conditions would exclude diagnosis of SS and participation in SS studies or therapeutic trials 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
Sarcoidosis
Amyloidosis
Graft-versus-host disease
IgG4-related disease

References:

Daniels TE, Cox D, Shiboski CH, et al. Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren's syndrome among 1,726 registry participants. Arthritis Rheum 2011; 63:2021.
Whitcher JP, Shiboski CH, Shiboski SC, et al. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry. Am J Ophthalmol 2010; 149:405.
Van Bijsterveld OP. Diagnostic tests in the Sicca syndrome. Arch Ophthalmol 1969; 82:10.
Navazesh M, Kumar SK, University of Southern California School of Dentistry. Measuring salivary flow: Challenges and opportunities. J Am Dent Assoc 2008; 139 Suppl:35S.

Reproduced with permission from: Shiboski CH, Shiboski SC, Seror R, et al. 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; 76(1):9-16. Copyright © 2017 BMJ Publishing Group Ltd.

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ACR/EULAR classification criteria for primary Sjögren's syndrome