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

https://doi.org/10.1016/j.jpeds.2007.09.019Get rights and content

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.

Section snippets

Methods

The study population comprised an inception cohort of all 256 patients with pSLE diagnosed at the Hospital for Sick Children, Toronto between March 1982 and January 2005. All patients met at least 4 of the American College of Rheumatology (ACR) classification criteria for SLE.21 All patients were followed in the pSLE clinic, and beginning in 1988, all data were prospectively collected and entered into a dedicated SLE database. Data obtained before 1988 was retrospectively obtained from chart

Demographic Data

The cohort consisted of 256 patients (211 females and 45 males [ratio, 4.7:1]), with a mean age at diagnosis of 13.1 ± 3.17 years (range, 3 to 18 years). The mean follow-up period was 3.5 ± 3 years (range, 0 to 16.2 years). A total of 81 of the 188 (43%) patients with Tanner stage information at diagnosis were diagnosed with SLE before puberty. There was no statistically significant difference in the female:male ratio of patients diagnosed prepubertally and that in those diagnosed

Discussion

SLE is a chronic autoimmune disease that affects both children and adults, with onset in childhood in approximately 20% of cases. Despite numerous reports of SLE in adults, in the past 10 years there have been fewer than 15 reports on the clinical and laboratory features of patients with onset of SLE in the pediatric age group and no large single-center inception cohort.3, 5, 6, 10, 14, 15, 29, 30, 31, 32 Although adult studies report a female:male ratio of approximately 10:1,33, 34, 35 in our

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