Long-term outcomes of childhood-onset systemic lupus erythematosus.

Mirguet, A; Aeschlimann, F A; Lemelle, I; Jaussaud, R; Decker, P; Moulinet, T; Mohamed, S; Quartier, P; Hofer, M; Boyer, O; Belot, A; Hummel, A; Costedoat-Chalumeau, N; Bader-Meunier, B

Mirguet, A; Aeschlimann, F A; Lemelle, I; Jaussaud, R; Decker, P; Moulinet, T; Mohamed, S; Quartier, P; Hofer, M; Boyer, O; Belot, A; Hummel, A; Costedoat-Chalumeau, N; Bader-Meunier, B.

Afiliação

Mirguet A; Department of Pediatric Nephrology, Children Hospital, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.
Aeschlimann FA; Department of Pediatric Immunology, Hematology and Rheumatology, Necker Hospital, AP-HP, National Reference Centre for Rheumatic and Autoimmune Diseases in Children, RAISE, Paris and Lyon, France, Institut IMAGINE, Université Paris-Cité, Paris, France.
Lemelle I; Department of Rheumatology, University Children's Hospital Basel, Basel, Switzerland.
Jaussaud R; Department of Pediatric Onco-hematology, Children Hospital, University Hospital of Nancy, Vandoeuvre-lès-Nancy, France.
Decker P; Department of Internal Medicine and Clinical Immunology, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.
Moulinet T; Department of Internal Medicine and Clinical Immunology, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.
Mohamed S; Department of Internal Medicine and Clinical Immunology, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.
Quartier P; University of Lorraine, UMR 7365 CNRS, IMoPA, VandÅuvre-lès-Nancy, France.
Hofer M; Department of Internal Medicine and Clinical Immunology, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.
Boyer O; Department of Pediatric Immunology, Hematology and Rheumatology, Necker Hospital, AP-HP, National Reference Centre for Rheumatic and Autoimmune Diseases in Children, RAISE, Paris and Lyon, France, Institut IMAGINE, Université Paris-Cité, Paris, France.
Belot A; Rheumatology, Immunology and Allergology Unit, Department of Pediatrics, Vaudois University Hospital, Lausanne, Switzerland.
Hummel A; Department of Pediatric Nephrology, MARHEA reference center, Necker Enfants maladies Hospital, AP-HP, Imagine Institute, Paris Cité University, Paris, France.
Costedoat-Chalumeau N; Pediatric Nephrology, Rheumatology, Dermatology Department, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 Bd Pinel, 68677, Bron Cedex, Franc; The International Center of Research in Infectiology, Lyon University, INSERM U1111, CNRS UMR 5308, ENS, UCBL, France, Lyon.
Bader-Meunier B; Department of Nephrology, Hospital Necker, AP-HP, Paris, France.

Rheumatology (Oxford) ; 2024 Jul 15.

Article em En | MEDLINE | ID: mdl-39008948

ABSTRACT

ABSTRACT

OBJECTIVE:

Data on the long-term outcome of patients with childhood-onset Systemic Lupus Erythematosus (cSLE) are scarce. Aims of this study were to describe the long-term outcomes of cSLE and to identify factors associated with the development of damage and persistent disease activity.

METHODS:

We conducted a retrospective multicentre study using data from the PEDIALUP registry of the Juvenile Inflammatory Rheumatism (JIR) cohort database. Demographic characteristics, clinical manifestations, laboratory, radiological, histological and treatment data were collected from medical records during follow-up.

RESULTS:

A total of 138 patients with cSLE, diagnosed between 1971 and 2015, were included. With a median follow-up of 15.4 [9.6-22.4] years, 51% of patients had a SLICC-Damage Index score ≥ 1 at last follow-up with the musculoskeletal, cutaneous, renal, neurological, and cardiovascular damage being the most common manifestations. The proportion of patients with a SLICC-DI score ≥ 1 increased significantly with the duration of the follow-up (p< 0.001). On multivariate analysis, duration of follow-up was associated with increased risk of cumulative damage (OR 1.08, 95% CI 1.01, 1.15, p= 0.035). At the last visit, 34% of patients still had active disease with a SLEDAI score of ≥ 6. On multivariate analysis, Sub-Saharan African ethnicity was associated with 7-fold increased odds of having active disease at the last visit compared with Caucasians (OR 7.44, 95% CI 2.24, 24.74, p= 0.0002).