2017 ACR–EULAR Classification Criteria for Primary Sjögren’s Syndrome

A diagnosis of primary Sjögren’s syndrome is often made on the basis of a classic triad of symptoms: dryness of the mouth and eyes, fatigue, and pain. Systemic complications, which are present in 30 to 40% of patients, may provide the first clues to the disease.

2017 ACR–EULAR Classification Criteria for Primary Sjögren’s Syndrome
On the basis of the listed classification criteria, a diagnosis of primary Sjögren’s syndrome is defined as a score of 4 or more. These criteria apply to patients who have at least one symptom of ocular or oral dryness or the presence of systemic manifestations suggestive of primary Sjögren’s syndrome. Exclusion criteria include active hepatitis C virus infection on polymerase-chain-reaction assay, radiotherapy of the cervical spine, sarcoidosis, graft-versus-host disease, receipt of anticholinergic drugs, and IgG4-related disease. ACR denotes American College of Rheumatology, EULAR European League against Rheumatism, SICCA Sjögren’s International Collaborative Clinical Alliance, and SSA anti–Sjögren’s syndrome–related antigen A.

Item Description Score
Focus score of ≥1 A score determined by the number of mononuclear-cell infiltrates containing ≥50 inflammatory cells per 4 mm2 of minor labial salivary gland obtained on biopsy 3
Presence of anti-SSA antibodies* Measured in serum; only anti-Ro60 antibodies have to be considered; isolated anti-Ro52 antibodies are not specific for Sjögren’s syndrome 3
SICCA ocular staining score of ≥5 A score determined by an ophthalmologist on the basis of examination with fluorescein and lissamine green staining; scores range from 0 to 12, with higher scores indicating greater severity 1
Schirmer test of ≤5 mm per 5 min An assay for measuring tear production by inserting filter paper on conjunctiva in the lower eyelid and assessing the amount of moisture on the paper 1
Unstimulated whole salivary flow of ≤0.1 ml per min An assay for measuring the rate of salivary flow by collecting saliva in a tube for at least 5 min after the patient has swallowed 1
Total score 9

* Positive serologic results for anti-SSB/La antibodies in the absence of anti-SSA/Ro antibodies is not specific and is no longer considered to be a criterion for the diagnosis.
 
 
References:

  1. Mariette X, Criswell LA. Primary Sjögren’s Syndrome. N Engl J Med. 2018 Mar 8;378(10):931-939. [Medline]
  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. Ann Rheum Dis. 2017 Jan;76(1):9-16. [Medline]

 
Created Jul 10, 2018

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