Relationship between damage and mortality in juvenile-onset systemic lupus erythematosus: Cluster analyses in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER)
Torrente-Segarra, V.; Monte, T. C. Salman; Rúa-Figueroa, I.;
de Uña-Álvarez, Jacobo; Balboa Barreiro, Vanesa; López-Longo, F. J.; Galindo-Izquierdo, M.; et al.,
Objectives: To identify patterns (clusters)of damage manifestation within a large cohort of juvenile SLE (jSLE)patients and evaluate their possible association with 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 manifestation were identified and compared. 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 number of individuals with damage (22.3% vs. 100% in Clusters 2 and 3, P < 0.001); Cluster 2 (14.5% of patients)was characterized by renal damage in 60% of patients, significantly more than Clusters 1 and 3 (P < 0.001), in addition to increased 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 by which patients were sorted into clinically meaningful clusters. We found two clusters of jSLE with important clinical damage that were associated with higher rates of mortality, especially for the cluster of patients with predominant renal damage. Physicians should be particularly vigilant to the early prevention of damage in this subset of jSLE patients with kidney involvement. © 2018 Elsevier Inc.
Type of Publication:
Cluster analysis; Juvenile Systemic Lupus Erythematosus; RELESSER; Organ damage; Mortality Journal:
Seminars in Arthritis and Rheumatism
Q1 5/31 h-index 5.072 (JCR2018)
Dr. Pego-Reigosa is supported by grant 316265 (BIOCAPS) from the European Union 7th Framework Program ( FP7/REGPOT-2012-2013.1 ). The FIS Grant PI11/02857 (Instituto Carlos III, Fondos FEDER ) has supported this work. Francisco Javier López-Longo has received speaker fees from Abbvie, Roche Farma, Bristol-Myers Squibb, Pfizer, UCB, MSD, Actelion. Francisco Javier López Longo has received research funding from Abbvie and GSK. The RELESSER Registry was funded by grants from GSK, Roche, UCB and Novartis. The other authors have declared no conflicts of interest. All authors have approved the final manuscript.