Outcome of lupus nephritis in childhood onset SLE in North and Central India: single-centre experience over 25 years.

Abstract

INTRODUCTION:

Childhood SLE (cSLE) has a higher prevalence of lupus nephritis (LN), and there are ethnic variations in response to treatment as well as outcome of LN. There are limited data on long-term outcome of LN in cSLE from the Indian subcontinent.

METHODS:

Retrospective analysis of case records of patients with cSLE (satisfying revised American College of Rheumatology (ACR) 1997 criteria for diagnosis) and age of onset <18 years was conducted from 1989 to 2013. Data on clinical features, renal involvement and biopsy findings, treatment, renal outcome, damage accrual and mortality were collected. End-stage renal disease (ESRD) was defined as the need for renal replacement therapy. Actuarial ESRD-free survival was studied as the primary outcome measure using Kaplan-Meier analysis.

RESULTS:

Among 205 children with cSLE, 134 (121 girls) had evidence of LN. The mean age at disease onset was 13.7 ± 3.5 years and the mean disease duration at presentation was 1.9 ± 2.5 years. Kidney biopsy was available for 92 patients, and histology included: 13 (14.2%) Class II, 24 (26%) Class III, 43 (46.7%) Class IV and 12 (13.1%) Class V LN. The mean follow-up period was 6.75 ± 5.7 years. At last visit, 81 (60.4%) children were in complete remission, 28 (20.9%) were in partial remission, 15 (11.2%) still had active nephritis and 10 (7.4%) had progressed to ESRD. Almost two-thirds (62.9%) of patients experienced lupus flares, and mean flare rate was 0.09 flares/patient follow-up year. Fifty-six (43.8%) children accrued damage and the mean Systemic Lupus International Collaborating Clinics (SLICC)/ACR damage score was 0.79 ± 1.13. Actuarial ESRD-free survival at five, 10 and 15 years was 91.1%, 79% and 76.2%, and five-, 10- and 15-year renal survival was 93.8%, 87.1% and 84%, respectively. Although multiple factors individually predicted poor outcome (death/ESRD), only raised serum creatinine at onset (R square = 0.65, p ≤ 0.0001) and damage accrual (R square = 0.62, p ≤ 0.0001) remained significant on multivariate analysis. Eleven (8.2%) children died during the follow-up period, and infections were the leading cause of mortality.

CONCLUSIONS:

Long-term outcome of LN in cSLE in our cohort was better than previous reports from India. However, a high rate of major infection still remains the leading cause of mortality.

• Authors: P Srivastava, B Abujam, R Misra, A Lawrence, V Agarwal, A Aggarwal
• Journal: Lupus (Lupus) Vol. 25 Issue 5 Pg. 547-57 (Apr 2016) ISSN: 1477-0962 [Electronic] England
• PMID: 26637291 (Publication Type: Journal Article)
• Copyright: © The Author(s) 2015.

Chemical References

• Anti-Infective Agents

Topics

• Adolescent
• Age of Onset
• Anti-Infective Agents (therapeutic use)
• Biopsy
• Cause of Death
• Chi-Square Distribution
• Child
• Communicable Diseases (drug therapy, epidemiology, mortality)
• Disease Progression
• Disease-Free Survival
• Humans
• India (epidemiology)
• Kaplan-Meier Estimate
• Kidney Failure, Chronic (diagnosis, epidemiology, mortality, therapy)
• Logistic Models
• Lupus Nephritis (diagnosis, epidemiology, mortality, therapy)
• Male
• Multivariate Analysis
• Remission Induction
• Retrospective Studies
• Risk Factors
• Time Factors
• Treatment Outcome