Wednesday, 18 Sep 2019

You are here

New EULAR/ACR Classification Criteria for SLE

The European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) have jointly developed new classification criteria for systemic lupus erythematosus (SLE); prompted by the need for criteria that were both highly sensitive and specific.  The net result is improved sensitivity and specificity, but the use of positive ANA requirement along with a longer list of weighted criteria ensures its utility in SLE research (including early or latent SLE), but not clinical practice.

This international initiative from many lupus research centers had four phases.

  1. Evaluation of antinuclear antibody (ANA) as an entry criterion
  2. Criteria reduction by Delphi and nominal group technique exercises
  3. Criteria definition and weighting
  4. Refinement of weighting and threshold scores in a new cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.

The process included 23 expert centers, with each contributing up to 100 SLE patients and non-SLE patients. Diagnoses were verified by 3 independent reviewers for 1,193 SLE and 1,059 non-SLE patients. 500 randomly selected SLE and non-SLE patients formed the derivation cohort and the remainder the validation cohort. 

Past criteria were either too sensitive (1982, revised 1997) or too sensitive with a loss of specificity (SLICC 2012). Thus the new criteria set out to improve both the sensitivity and specificity.

 

ACR 1997

SLICC 2012

New criteria 2019

Derivation

  Sensitivity %

85

97

98

  Specificity %

95

90

96

Validation

  Sensitivity %

83

97

96

  Specificity %

93

84

93

The new 2019 EULAR/ACR classification criteria for SLE requires a positive ANA as obligatory entry criterion. Other criteria were chosen from 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) categories, and weighted from 2 to 10.

Patients with ≥10 points are classified as having SLE

In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.

The following algorithm outlines the weighted point system used to classify a patient as SLE:

 

Disclosures: 
The author has no conflicts of interest to disclose related to this subject

Add new comment

More Like This

FDA Grants Breakthrough Status for Potential Lupus Nephritis Drug

Obinutuzumab (Gazyva) has been granted Breakthrough Therapy Designation (BTD) by the U.S. Food and Drug Administration (FDA) for use in adults with lupus nephritis (LN). The drug made by Genentech, is going forward based on the Phase II NOBILITY study in adult patients with proliferative lupus nephritis (LN). Currently, there are no FDA-approved medicines for lupus nephritis.

Riociguat Fails in Systemic Sclerosis-Associated Digital Ulcers

Riociguat is an oral, selective soluble guanylate cyclase stimulator that has been studied in patients with digital ulcers (DU) due to systemic sclerosis (SSc) but study results show that short term (16 weeks) riociquat therapy does not sufficiently reduce the DU burden in SSc patients.

Nintedanib FDA Approved for Scleroderma Lung Disease

Last Friday, the US Food and Drug Administration approved Ofev (nintedanib) to slow the rate of decline in pulmonary function in adults with interstitial lung disease associated with systemic sclerosis or scleroderma, called SSc-ILD. ILD as a complication of SSc may lead to progressive loss of lung function and may be associated with a significant mortality risk. Prior to the approval of Olev, there were no FDA approved drugs for SSc-ILD.

With Autoimmunity, Checkpoint Inhibitors Can Be Used

Among patients with pre-existing autoimmune diseases who developed cancer and were treated with immune checkpoint inhibitors (ICI), flares of the underlying disease and other immune-related adverse events were common, a retrospective study conducted in France showed.

Sjogren's Syndrome at Risk for Psychiatric Disorders

A population-based claims study from Taiwan shows significantly increased incidences of depressive disorder, anxiety disorder, and sleep disorder in patients with primary Sjögren’s syndrome (pSS).