Clinical and Laboratory Characteristics and Long-Term Outcome of Pediatric Systemic Lupus Erythematosus: A Longitudinal Study

• Published in: The Journal of pediatrics 2008-04, Vol.152 (4), p.550-556
• Main Authors: Hiraki, Linda T., MD, Benseler, Susanne M., MD, Tyrrell, Pascal N., MSc, Hebert, Diane, MD, FRCPC, Harvey, Elizabeth, MD, FRCPC, Silverman, Earl D., MD, FRCPC
• Format: Article
• Language: English
• Subjects: Adolescent; Age of Onset; Antimalarials - therapeutic use; Autoantibodies - blood; Azathioprine - therapeutic use; Biological and medical sciences; Central Nervous System Diseases - etiology; Child; Child, Preschool; Disease Progression; Drug Utilization - statistics & numerical data; Female; General aspects; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Kaplan-Meier Estimate; Kidney Diseases - etiology; Longitudinal Studies; Lupus Erythematosus, Systemic - blood; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - drug therapy; Lupus Erythematosus, Systemic - mortality; Male; Medical sciences; Pediatrics; Prednisone - therapeutic use; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2007.09.019

Objectives To determine the frequency and characteristics of clinical signs, symptoms, laboratory findings, and medication use in children with pediatric systemic lupus erythematosus (pSLE) at presentation and during the course of the disease, and to examine correlations among disease manifestations, disease activity, and damage over time. Study design The study involved an analysis of medical records and the SLE database of an inception cohort of 256 patients with pSLE (female:male ratio, 4.7:1). Results The most common clinical manifestations were arthritis (67%), malar rash (66%), nephritis (55%), and central nervous system (CNS) disease (27%). At diagnosis, patients with both renal and CNS disease had the highest SLE Disease Activity Index (SLEDAI) scores ( P < .0001), but these scores were similar to those of the total group at 1 year ( P = .11). Patients who developed renal and CNS disease more than 1 year after diagnosis had higher SLEDAI scores at disease onset. Some 34% of patients had Systemic Lupus International Collaborative Clinics Damage Index (SLICC-DI) scores >1 at a mean follow-up of 3.5 years. A greater proportion of patients with renal and CNS disease had SLICC-DI scores of >1, and these patients had higher mean scores compared with patients without major organ involvement (70% vs 11% [ P < .0001] and 1.4 vs 0.1 [ P < .0001], respectively). Conclusions Most of the patients in our cohort exhibited major organ involvement. These patients had the highest SLEDAI scores at diagnosis, which normalized at 1 year but preceded development of renal and CNS disease. The average SLICC-DI score was lower than that previously reported in patients with pSLE.