Background: Background/Purpose Systemic lupus erythematosus (SLE) is a prototype autoimmune disease. The most widely used classification criteria for SLE were those developed by the American College of Rheumatology (ACR) in 1982. The Systemic Lupus Collaborating Clinics (SLICC) revised the ACR SLE classification criteria and validated new criteria in adults.
Objectives To assess sensitivity and specificity of revised and validated new SLICC SLE classification criteria in a cohort of Juvenile SLE (JSLE).
Methods The SLICC criteria rule for SLE classification requires: 1) four criteria, with at least one clinical criterion and one immunological criterion or 2) lupus nephritis alone in the presence of ANA or anti-DNA antibodies. Cases were JSLE patients who have attended a single tertiary center in the past 10 years. JSLE had been diagnosed on clinical and immunological grounds by experienced pediatric rheumatologist. Controls were patients with rheumatic diseases other than SLE: Juvenile Idiopathic Arthritis (JIA); Juvenile Dermatomyositis (JDM), Autoimmune Hepatitis (AH), Juvenile Systemic Sclerosis (JSS), ANCA-associated vasculitis (AAV) and Henoch Schönlein Purpura (HSP). Clinical, immunological and pathological were reviewed from prospectively developed databases and medical records in order to establish the number and frequency of new criteria. Statistics included overall sensitivity and specificity. McNemar`s test was used to assess differences between ACR criteria and new current criteria.
Results Cases: 107 patients with JSLE were included (F: 89 M: 18), age at onset: 12 (3-16) yo. Controls: 124 patients with JIA (36 patients); JDM (28), AH (28), JSS (10), AAV(12), HSP (10) F:95 M: 29, age at onset: 11 (2-16)yo. SLICC SLE criteria sensitivity was 100 % vs 86% ACR criteria, while specificity was 98% vs 96% (p=0.009). Six patients with a clinical diagnosis of JSLE were correctly classified by SLICC but not by ACR criteria.
Conclusions The SLICC new criteria performed better than the ACR 1997 criteria in a cohort of patients with JSLE. Validation of these criteria in a wider, multiethnic cohort of patients with JSLE is necessary.
Disclosure of Interest None Declared