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Published on: November 1, 2015
Patterns and influence of familial autoimmunity in pediatric systemic lupus erythematosus
Heather M Walters1, Nancy Pan, Lakshmi N Moorthy
1Komansky Center for Children's Health/NY Weill Cornell Medical Center, New York, NY, USA. waltersh@hss.edu.
Insights
Autoimmune diseases are common in families of children with systemic lupus erythematosus (SLE). However, a family history of autoimmune disease did not predict SLE severity in these pediatric patients.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Genetics
Background:
- Autoimmune diseases (AD) are frequently observed in relatives of adult systemic lupus erythematosus (SLE) patients.
- Limited data exists on familial inheritance patterns in pediatric SLE cases.
Purpose of the Study:
- To determine the prevalence and types of AD in relatives of pediatric SLE patients.
- To compare SLE severity in children with and without a family history of AD.
- To assess the influence of baseline demographics on pediatric SLE severity.
Main Methods:
- Retrospective chart review of 69 pediatric-onset SLE patients.
- Analysis of family history of AD among first, second, and third-degree relatives.
- Comparison of SLE severity based on family history and demographic factors.
Main Results:
- 42% of pediatric SLE patients had at least one relative with AD; 32% had a first-degree relative with AD.
- The most common familial ADs were SLE (21%) and thyroid disease (15%).
- Children without a family history of AD were more likely to have severe SLE; earlier onset correlated with increased severity.
Conclusions:
- A significant prevalence of AD exists in families of children with SLE.
- Family history of AD did not correlate with increased SLE severity in pediatric patients.
- Further research with larger cohorts is needed to understand inheritance patterns and factors influencing pediatric SLE severity.
Background:
A high prevalence of autoimmune disease (AD) has been documented in relatives of adult patients with systemic lupus erythematosus (SLE). However, data on familial inheritance patterns in pediatric SLE patients is scarce.
Findings:
The charts of 69 patients with pediatric-onset SLE were reviewed retrospectively. The primary aim was to describe the prevalence and types of AD in relatives of children with SLE. The secondary aims were: 1) to compare severity of SLE in children with and without relatives affected by AD, and 2) to evaluate the impact of baseline demographics on severity of SLE in subjects. At diagnosis, 42% of subjects had one or more first, second, or third degree relative(s) with AD; and 32% of subjects had one or more first degree relative(s) with AD. The most common diseases in relatives of children with SLE were SLE (21%) and thyroid disease (15%). Subjects with no family history of AD were more likely to have severe SLE. SLE severity in subjects did not differ by gender. Children presenting with SLE at an earlier age were found to have more severe disease.
Conclusions:
This study demonstrated a high prevalence of AD in families of children with SLE, although a family history of AD did not correlate with more severe SLE in subjects. Future larger studies are necessary to elucidate patterns of familial inheritance and baseline patient characteristics that may affect severity of disease in pediatric SLE.
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