Childhood-onset XXXX in Singapore: clinical phenotypes, disease activity, damage, and autoantibody profiles.

@entity1 @entity2530 @entity8 @entity231 @entity1252 @entity1200 @entity2166 @entity204 @entity792 @entity479
UNASSIGNED: Childhood-onset @entity1252 (cSLE) is a multisystem @entity792 characterized by @entity1200 affecting @entity1 less than 18 years old. One-fifth of @entity1252 cases are diagnosed during childhood. cSLE presents differently from adults and has a more severe and aggressive course. We describe the clinical and antibody profiles in our cSLE Singapore cohort. All cSLE @entity1 who satisfied the 1997 American College of Rheumatology diagnostic criteria were captured in our lupus registry from January 2009 to January 2014. Data including demographic, cumulative clinical, serologic data, and damage indices were collected. @entity2166 ( @entity2166 ) was used to summarize disease activity over multiple visits. Cluster analysis using non-hierarchical K-means procedure was performed on eight selected antibodies. The 64 @entity1 (female:male ratio 5:1; Chinese 45.3%, Malay 28.1%, Indian 9.4%, and other races 17.2%) had a mean onset age of 11.5 years (range 2.1-16.7) and mean age at diagnosis was 11.9 years (range 2.6-18.0). Our study demonstrated differences in clinical manifestations for which hematologic involvement was the most common manifestation with @entity8 and uncommon neurologic manifestation as compared to other cSLE cohorts reported in our region. Antibody clusters were identified in our cohort but their clinical association/discrimination and outcome prediction required further validation study. Outcomes of our cohort in regard to disease activity after therapy and organ damages were comparable if not better to other cSLE cohorts elsewhere. @entity479 -related damage, including symptomatic @entity2530 and @entity231 , were not uncommon locally. Infection remains the major cause of @entity204 for the continent. Nevertheless, the five year survival rate of our cohort (98.4%) was high.
