Article Text
Abstract
Objectives Identify patterns (clusters) of damage manifestations within a large cohort of patients with juvenile-onset SLE (jSLE) and evaluate their potential association with mortality.
Methods Multicenter, descriptive and cross-sectional study of a cohort of 345 patients with jSLE (age at SLE diagnosis <18 years) from the Lupus Registry of the Spanish Society of Rheumatology (RELESSER). Organ damage was determined by using the SLICC/ACR damage index (SDI). By using cluster analysis, groups of patients with similar patterns of damage manifestations were identified and compared among them.
Results Mean age (years)±S.D. at diagnosis was 14.2±2.89, 88.7% were female and 93.4% were Caucasian. Mean SLICC/ACR DI±S.D. was 1.27±1.63. A total of 12 (3.5%) patients died. Three damage clusters were identified: Cluster 1 (72.7% of patients) presented a lower amount of individuals with damage (22.3% vs 100% in clusters 2 and 3, p<0.001). Cluster 2 (14.5% of patients) was featured by renal damage in 60% of patients, significantly more frequent than clusters 1 and 3 (p<0.001), alongside more ocular, cardiovascular and gonadal damage. Cluster 3 (12.7%) was the only group with musculoskeletal damage (100%), significantly higher than in clusters 1 and 2 (p<0.001). The overall mortality rate in cluster 2 was 2.2 times higher than that in cluster 3 and 5 times higher than that in cluster 1 (p<0.017 for both comparisons).
Conclusions In a large cohort of jSLE patients, renal and musculoskeletal damage manifestations were the two dominant forms of damage to sort patients into clinically meaningful clusters. We found two clusters of jSLE with important clinical damage that were associated to higher rates of mortality, especially for the cluster of patients with predominant renal damage. Physicians should pay special attention to the early prevention of damage in this particular subset of jSLE patients with kidney involvement.
Disclosure of Interest None declared