Objectives To identify patterns (clusters) of damage manifestations within a large cohort of juvenile SLE (jSLE) patients and evaluate the potential association of these clusters with a higher risk of mortality.
Methods This is a multicentre, descriptive, cross-sectional study of a cohort of 345 jSLE patients from the Spanish Society of Rheumatology Lupus Registry. Organ damage was ascertained using the Systemic Lupus International Collaborating Clinics Damage Index. Using cluster analysis, groups of patients with similar patterns of damage manifestations were identified.
Results Mean age at diagnosis 14.2±2.89, 88.7% were female and 93.4% were Caucasian. A total of 12 (3.5%) patients died, mean SLICC/ACR DI 1.27±1.63. Three damage clusters were identified:
Cluster 1 (72.7% of patients) showed damage in only 22.3% of patient, but no significant domain was involved.
Cluster 2 (14.5%) was featured by renal damage in 60% of patients, ocular damage in 54%, cardiovascular damage in 20% and gonadal failure in 14%, all significantly higher than clusters 1 and 3 (p<0.001). All patients scored for some damage in SLICC/ACR DI index, with a mean of 2.90±1.54 and mean affected domains of 1.86±0.93.
Cluster 3 (12.7%) was the only group with musculoskeletal damage (100%), clearly higher than clusters 1 and 2. All patients scored for some damage in SLICC/ACR DI index, with a mean of 2.66±1.87 and mean affected domains of 1.89±1.18.
The overall mortality rate of patients in clusters 2 and 3 was higher than in cluster 1 (p<0.05) and significantly higher in cluster 2 (2.2x times than cluster 3 and 5x times than cluster 1) (See table 1).
Conclusion In a large cohort of jSLE patients, we found one cluster with several damage domains involved that we consider clinically meaningful. Another cluster with important musculoskeletal damage manifestations and another cluster with no clinically significant damage at all were also found. These two clusters of jSLE with important clinical damage were found to be associated to higher rates of mortality, specially for the cluster involving renal, ocular, cardiovascular and gonadal domains. Physicians should pay special attention to the early prevention of damage in these particular subsets of patients.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.