Journal Article

In an early SLE cohort the ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria classify non-overlapping groups of patients: use of all three criteria ensures optimal capture for clinical studies while their modification earlier classification and treatment

Adamichou, Christina, Nikolopoulos, Dionysis, Kalogiannaki, Eleni, Genitsaridi, Irini, Bortoluzzi, Alessandra, Gergianaki, Irini, Bertsias, George K, Sidiropoulos, Prodromos, Boumpas, Dimitrios T, Papastefanakis, Emmanouil, Fanouriakis, Antonis

2020

Overview

ObjectivesClassification criteria are biased towards classifying long-standing disease. We compared the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)-2019, Systemic Lupus International Collaborating Clinics (SLICC)-2012 and ACR-1997 criteria in an early (median 48 months) systemic lupus erythematosus (SLE) cohort.MethodsPatients diagnosed with SLE (n=690) or control diseases (n=401). Sensitivity, specificity of the criteria and time-to-classification were calculated. Modified classification algorithms were derived from a random 80% and validated in the remaining 20% of the dataset running multiple iterations.ResultsAt last assessment, sensitivities of ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria were 85.7%, 91.3% and 88.6%, with specificities 93.0%, 93.8% and 97.3%, respectively. Both SLICC and EULAR/ACR enabled earlier classification. Only 76.7% of patients with SLE met all three criteria suggesting non-overlapping groups. Notably, unclassified patients had high prevalence of British Isles Lupus Assessment Group moderate/severe manifestations (43.3%–60%) and SLICC/ACR organ damage (30%–50%). At diagnosis, criteria missed 25.6%–30.5% of patients. Modification of EULAR/ACR and SLICC algorithms to include hypocomplementaemia and/or positive anti-phospholipid antibodies as alternative entry criterion, and/or allow classification with fewer clinical criteria from multiple organs, increased their sensitivity at diagnosis (median 82.0% and 86.2%) and overall (93.7% and 97.1%) with modest decreases in specificity. Importantly, patients who were still missed by the modified criteria had lower incidence of major organ involvement, use of immunosuppressive/biological therapies and organ damage.ConclusionsThe SLICC and EULAR/ACR are more sensitive than the ACR and the EULAR/ACR criteria have superior specificity in early SLE, although patients with significant disease can be missed. Combination and/or modification of the classification algorithms may enhance their sensitivity, allowing earlier classification and treatment of more patients with high disease burden.

• Publisher: BMJ Publishing Group Ltd and European League Against Rheumatism,Elsevier Limited
• Subject: Adult / Algorithms / Antiphospholipid antibodies / autoimmune diseases / Classification / Clinical medicine / Diagnosis / Disease / Female / Humans / Immunosuppressive agents / Lupus / Lupus Erythematosus, Systemic - classification / Lupus Erythematosus, Systemic - diagnosis / Male / Middle Aged / outcomes research / Patients / Phospholipids / Retrospective Studies / Rheumatism / Rheumatology / Rheumatology - methods / Rheumatology - standards / Sensitivity and Specificity / Severity of Illness Index / Studies / Symptom Assessment - methods / Symptom Assessment - standards / Systemic lupus erythematosus