Toward new criteria for systemic lupus erythematosus—a standpoint

Abstract

While clearly different in their aims and means, classification and diagnosis both try to accurately label the disease patients are suffering from. For systemic lupus erythematosus (SLE), this is complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. Hallmarks of SLE are the presence of antinuclear antibodies (ANA), and multiple immune-mediated organ symptoms that are largely independent. In an attempt to overcome limitations of the current sets of SLE classification criteria, a new fourphase approach is being developed, which is jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This review attempts to delineate the performance of the current sets of criteria, the reasons for the decision for classification, and not diagnostic, criteria, and to provide a background of the current approach taken. Lupus (2016) 25, 805-811.

How Do Patients With Newly Diagnosed Systemic Lupus Erythematosus Present? A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria

“…In our cohort of 616 patients, the 1997 ACR criteria demonstrated a sensitivity of 66.1% and a specificity of 91.6%, and the 2012 SLICC criteria demonstrated a sensitivity of 83.5% and a specificity of 82.4% for early diagnosis . As a result, 132 patients with a clinical diagnosis of SLE (33.9%) were not classified as having SLE according to the ACR criteria, and 64 with a clinical diagnosis of SLE (16.5%) did not fulfill the SLICC classification criteria.…”

“…Despite differences in the aims and means of classification and diagnosis, classification criteria enhance physicians’ ability to accurately identify and recognize SLE . The goals of the current multicenter study were to 1) evaluate the characteristics of patients with early SLE compared to non‐SLE patients, 2) identify manifestations at disease onset that may support the early diagnosis of SLE, and 3) inform the development of new classification criteria, which could potentially and accurately identify more patients in the early stages of SLE.…”

2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus

“…Using a methodologic approach based on measurement science the criteria were developed in four phases : 1) criteria generation, 2) criteria reduction, 3) criteria definition and weighting, and 4) refinement and validation (Figure ). The whole initiative was overseen by a 12‐member steering committee (MA, KHC, DID, MM, RR‐G, JSS, DW, DTB, DLK, DJ, TD, and SRJ) nominated by EULAR and the ACR in equal numbers, based on SLE and/or methodologic experience and previous involvement in international projects.…”

ANA-Negative Presentation of SLE in Man with Severe Autoimmune Neutropenia

“…Currently, the ACR and EULAR are working together to develop new classification criteria for SLE [24]. Using a four-phase process, this project seeks to review ANA sensitivity and specificity, weigh the importance of entry and additive criteria to eliminate redundancy and low-yield measures, and test the performance of these newly derived criteria against 1997 ACR and 2012 SLICC criteria.…”