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Semin Arthritis Rheum. 2019 Jun;48(6):1025-1029. doi: 10.1016/j.semarthrit.2018.09.005. Epub 2018 Sep 27.

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).

Author information

1
Rheumatology Department, Hospital General Hospitalet-Sant Joan DespíMoisèsBroggi, C/ Josep Molins 29-41, 08906 Hospitalet, Llobregat 08906, Spain. Electronic address: vtorrente@hsjdbcn.org.
2
Rheumatology Department, Parc de Salut Mar-IMIM, Barcelona, Spain.
3
Rheumatology Department, Doctor Negrín University Hospital of Gran Canaria, Las Palmas de Gran Canaria, Spain. Electronic address: iruafer@gobiernodecanarias.org.
4
Department of Statistics and OR, Vigo University, Vigo, Spain.
5
Rheumatology Department, Gregorio Marañón University Hospital, Madrid, Spain.
6
Rheumatology Department, Doce de Octubre University Hospital, Madrid, Spain. Electronic address: mgalindo@h12o.es.
7
Rheumatology Department, Sierrallana Hospital, Torrelavega, Spain. Electronic address: jaime.calvo@scsalud.es.
8
Rheumatology Department, Germans TríasiPujol University Hospital, Badalona, Spain. Electronic address: aolive.germanstrias@gencat.cat.
9
Rheumatology Department, Complexo Hospitalario Universitario de Vigo, Spain. Electronic address: coral.mourino.rodriguez@sergas.es.
10
Rheumatology Department, Navarra Hospital, Navarra, Pamplona, Spain.
11
Rheumatology Department, Príncipe de Asturias University Hospital, Madrid, Spain.
12
Rheumatology Department, Salamanca Clinic University Hospital, Salamanca, Spain.
13
Rheumatology Department, Complexo Hospitalario Universitario de Ourense, Ourense, Spain. Electronic address: eva.salgado@sergas.es.
14
Rheumatology Department, León Hospital, León, Spain.
15
Rheumatology Department, Marqués de Valdecilla University Hospital, Santander, Spain. Electronic address: rblanco@humv.es.
16
Rheumatology Department, Puertadel Hierro-Majadahonda Hospital, Madrid, Spain. Electronic address: jlandreu@arrakis.es.
17
Rheumatology Department Basurto Hospital, Basurto, Spain. Electronic address: OLAIABEGONA.FERNANDEZBERRIZBEITIA@osakidetza.net.
18
José Ángel, Rheumatology Department, Hospital Insular of Gran Canaria, Las Palmas de Gran Canaria, Spain.
19
RheumatologyDepartment, Tenerife Clinic Hospital, Tenerife, Spain.
20
Rheumatology Department, Virgen Macarena Hospital, Sevilla, Spain.
21
RheumatologyDepartment, Miguel Servet University Hospital, Zaragoza, Spain. Electronic address: apecondon@ono.com.
22
RheumatologyDepartment, Virgen de laArrixaca University Hospital, Murcia, Spain.
23
RheumatologyDepartment, La Paz University Hospital, Madrid, Spain. Electronic address: gemabonilla@ser.es.
24
Rheumatology Department, Complexo Hospitalario Universitario de Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) Group Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain. Electronic address: jose.maria.pego.reigosa@sergas.es.

Abstract

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.

KEYWORDS:

Cluster analysis; Juvenile Systemic Lupus Erythematosus; Organ damage, Mortality; RELESSER

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