Outcome of XXXX in childhood onset @entity1252 in North and Central India: single-centre experience over 25 years.

@entity1 @entity1310 @entity1252 @entity1988 @entity281 @entity1011 @entity204 @entity454
INTRODUCTION: Childhood @entity1252 (cSLE) has a higher prevalence of @entity1988 ( @entity1988 ), and there are ethnic variations in response to treatment as well as outcome of @entity1988 . There are limited data on long-term outcome of @entity1988 in cSLE from the Indian subcontinent. METHODS: Retrospective analysis of case records of @entity1 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. @entity454 ( @entity454 ) was defined as the need for renal replacement therapy. Actuarial @entity454 -free survival was studied as the primary outcome measure using Kaplan-Meier analysis. RESULTS: Among 205 @entity1 with cSLE, 134 (121 @entity1 ) had evidence of @entity1988 . 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 @entity1 , and histology included: 13 (14.2%) Class II, 24 (26%) Class III, 43 (46.7%) Class IV and 12 (13.1%) Class V @entity1988 . The mean follow-up period was 6.75        5.7 years. At last visit, 81 (60.4%) @entity1 were in complete remission, 28 (20.9%) were in partial remission, 15 (11.2%) still had active @entity1310 and 10 (7.4%) had progressed to @entity454 . Almost two-thirds (62.9%) of @entity1 experienced @entity1252 , and mean flare rate was 0.09 flares/ @entity1 follow-up year. Fifty-six (43.8%) @entity1 accrued damage and the mean Systemic Lupus International Collaborating Clinics (SLICC)/ACR damage score was 0.79        1.13. Actuarial @entity454 -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 ( @entity204 / @entity454 ), only raised serum @entity1011 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%) @entity1 died during the follow-up period, and @entity281 were the leading cause of mortality. CONCLUSIONS: Long-term outcome of @entity1988 in cSLE in our cohort was better than previous reports from India. However, a high rate of major @entity281 still remains the leading cause of mortality.
