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The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice
Published on: November 1, 2015
A longitudinal PRINTO study on growth and puberty in juvenile systemic lupus erythematosus
Marite Rygg1, Angela Pistorio, Angelo Ravelli
1Faculty of medicine, Department of Laboratory Medicine, Children’s and Women’s Health, Norwegian University of Science and Technology, Trondheim, Norway.
Insights
Juvenile systemic lupus erythematosus (SLE) significantly impacts growth and puberty, especially in males and younger females. High corticosteroid doses increase the risk of growth failure and delayed puberty in children with SLE.
Area of Science:
- Pediatric Rheumatology
- Endocrinology
- Growth and Development
Background:
- Juvenile systemic lupus erythematosus (jSLE) is a chronic autoimmune disease affecting children.
- Longitudinal data on growth and pubertal development in jSLE are crucial for management.
- Previous studies have indicated potential growth disturbances in jSLE patients.
Purpose of the Study:
- To collect longitudinal data on growth and pubertal development in a large, multinational prospective cohort of children with jSLE.
- To identify factors influencing growth and pubertal outcomes in this population.
Main Methods:
- A prospective, multi-national cohort study involving 331 patients with jSLE (≤18 years old) in active disease phase.
- Anthropometric data (height, BMI) were collected at four follow-up visits.
- Analysis included assessment of height z scores, BMI z scores, pubertal onset, and menarche, with statistical analysis of influencing factors.
Main Results:
- Significant reduction in height z scores over time in both genders, with males more affected.
- Body mass index z scores peaked at 6 months and remained elevated.
- Growth failure observed in 14.7% of females and 24.5% of males; delayed puberty in a significant proportion of both genders.
- Risk factors for growth failure included prior growth failure, younger age at first visit, and high cumulative corticosteroid dose.
Conclusions:
- Children with jSLE experience significant negative effects on height and pubertal development.
- Prepubertal and peripubertal children receiving >400 mg/kg cumulative corticosteroid dose are at higher risk.
- Early identification and intervention are necessary to mitigate long-term growth and pubertal sequelae.
Objective:
To obtain longitudinal data on growth/puberty in a large-scale, multi-national prospective cohort of juvenile systemic lupus erythematosus (SLE).
Methods:
Data from 331/557 (59.4%) patients ≤18 years old with juvenile SLE in active phase, with anthropometric data available at four follow-up visits, were studied.
Results:
There was a significant reduction in parent-adjusted height z score with time in females and males (p<0.0001), with a significant gender difference (p<0.0001) and with male height being most affected. Median body mass index z score peaked at 6 months and was still significantly above baseline after 26 months (p<0.01), with no gender difference. Standardised height reduction was inversely related to age at onset. Females with onset age <12 years had a median parent-adjusted height z score of -0.87 with no catch-up growth. At the end of the study, growth failure was seen in 14.7% of the females and 24.5% of the males. Height deflection (less than -0.25/year) was found in 20.7% of the females and 45.5% of the males. Delayed pubertal onset was seen in 15.3% and 24% of the females and males, respectively, and delayed/absent menarche was seen in 21.9%, while 36.1% of the females and 44% of the males had some degree of delayed pubertal development. Growth failure baseline determinants were previous growth failure (OR: 56.6), age at first visit ≤13.4 years (OR: 4.2) and cumulative steroid dose >426 mg/kg (OR: 3.6).
Conclusions:
The children at risk of having a negative effect on height and pubertal development are prepubertal and peripubertal children treated with >400 mg/kg cumulative dose of corticosteroids.
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