Comparison of patients with isolated cutaneous lupus erythematosus versus systemic lupus erythematosus with cutaneous lupus erythematosus as the sole clinical feature: A monocentric study of 149 patients

Abstract

BackgroundCutaneous lupus erythematosus (CLE) may present as an isolated entity or be classified as Systemic lupus erythematosus (SLE) by the presence of laboratory abnormalities including cytopenia, low complement levels and/or autoantibodies (CLE with laboratory SLE). ObjectiveTo compare isolated CLE and CLE with laboratory SLE and to validate an existing 3-items score with age <25 years (1 point), phototypes V-VI (1 point), antinuclear antibodies ≥1:320 (5 points) to predict the risk of progression from CLE to severe SLE. MethodsMonocentric cohort study including consecutive CLE patients. CLE with laboratory SLE was defined by 2019 ACR/EULAR classification criteria for SLE score of ≥ 10 points at baseline with CLE as the sole clinical feature. ResultsOf the 149 CLE patients, 20 had CLE with laboratory SLE. Median follow-up duration was 11.3 years (IQR: 5.1-20.5). Ten patients (7%) developed severe SLE. In survival analysis, the risk of progression to severe SLE was higher among CLE with laboratory SLE (HR=6.69, [95%CI: 1.93-23.14], p<0.001) compared to isolated CLE. In both groups, none of the patient with a risk score ≤ 2 developed severe SLE. LimitationsMonocentric study with limited number of patients ConclusionCLE with laboratory SLE patients have higher risk of progression to severe SLE than isolated CLE.