Differential manifestations of prepubescent, pubescent and postpubescent pediatric patients with systemic lupus erythematosus: A retrospective study of 96 Chinese children and adolescents

Abstract

BackgroundChildren represent 10-20% of all systemic lupus erythematosus (SLE) patients. Their clinical manifestations and outcomes vary with age. We aim to clarify the relationship between pubescent status and the clinical manifestations of pediatric SLE.MethodsIn this study, pediatric SLE patients were divided into three groups, based on age at disease onset (≦8, 8–13 & 13–18 years), defined as prepubescent, pubescent and postpubescent, respectively. Initial clinical manifestations and laboratory characteristics at diagnosis were analyzed.ResultsNinety-six patients were entered into the study: 8 had disease onset before age 8, while 49 were between 8–13 and 39 of them were 13–18. Female predominance was noted in all three groups (2.5-7.0:1). Postpubescents showed significantly more renal involvement and lymphopenia, along with lower levels of C3 and C4, when compared with prepubescents. They also showed significantly more lymphopenia when compared with pubescents. Pubescents showed significantly more renal involvement, leukopenia and lupus anticoagulant (LAC) positivity, along with lower C3 and C4 levels, when compared with prepubescents. Pubescents also showed significantly higher anti-Sm antibody positivity when compared with postpubescents. Prepubescents showed significantly more splenomegaly and anti-Jo-1 antibody positivity when compared with those of pubescents. The results showed that the disease activity (SLEDAI-2K score) correlated positively with age at disease onset and negatively with disease duration before diagnosis (p = 0.011).ConclusionsAge at disease onset is related to initial manifestations in pediatric SLE patients at our center. Certain parameters such as renal involvement, splenomegaly, low C3 level, low C4 level, lymphopenia, leukopenia, and anti-Sm & anti-Jo-1 antibody were found to be significantly different among the age groups. Renal involvement might be the key symptom that varies with age.

Features of 847 Childhood‐Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study

“…Previous retrospective studies suggested that age at disease onset (10)(11)(12)(13) and age at diagnosis (9) may influence disease expression in terms of initial clinical presentation, pattern of organ involvement and serological findings. The small sample size of these studies performed in North America, Europe and Asian cSLE populations (8)(9)(10)(11)(12)(13) precludes a definitive conclusion about their findings, particularly regarding the early-onset cSLE patients (<6 years).…”

“…The small sample size of these studies performed in North America, Europe and Asian cSLE populations (8)(9)(10)(11)(12)(13) precludes a definitive conclusion about their findings, particularly regarding the early-onset cSLE patients (<6 years). In addition, there is no published data on the relevance of age in a large cohort of Latin-American cSLE patients.…”

"Our Little Secret": pinpointing potential predators

“…Interpreting such definitions would seem to require clarification of prepubescent. The age of puberty can vary from person to person, but a recent article in a paediatric journal suggested "patients ≦ 8years old (prepubescent), [8][9][10][11][12][13] (pubescent), 13-18 (post-pubescent)" [7]. It is possible that these age ranges suit the country and the study, but they appear unduly low and it may be more typical to distinguish amongst those sexually attracted to infants from 0-5 years, (called Nepiophilia or infantophilia), children younger than 11 (Paedophilia, which may incorporate Nepiophilia), ages 11-14 (Hebephilia) and 15-19 (Ephebophilia) e.g.…”

Experience in Rituximab Administration in Children with Systemic Lupus Erythematosus: a Retrospective Study of the Case Series

“…Системная красная волчанка (СКВ) -системное ауто-иммунное заболевание с мультиорганным вовлечением, поражающее до 10-20% детей [1]. Клинические прояв-ления СКВ крайне вариабельны, течение зачастую мало предсказуемо; имеет тенденцию к более тяжелому течению при возникновении в детском возрасте [2][3][4].…”