Taxonomy for systemic lupus erythematosus with onset before adulthood

Area of Science:

• Rheumatology
• Pediatric Rheumatology
• Medical Nomenclature

Background:

• Systemic lupus erythematosus (SLE) is a chronic autoimmune disease.
• Lack of a consistent nomenclature for pediatric SLE cases complicates research and clinical practice.
• Existing terms for childhood-onset SLE vary widely in the literature.

Purpose of the Study:

• To establish a unified nomenclature for individuals meeting SLE classification criteria during childhood or adolescence.
• To address the variability in terminology used for pediatric SLE.

Main Methods:

• Comprehensive review of medical literature from 1960 to 2011 concerning pediatric SLE.
• Analysis of age thresholds and disease features differentiating childhood-onset from adult-onset SLE.
• Examination of nomenclature used for other chronic diseases with childhood and adult onset.

Main Results:

• Significant variability observed in age cutoffs (14-21 years) for defining SLE onset before adulthood, with 18 years being the most common.
• Commonly used synonyms included SLE (unspecified onset), childhood-onset SLE, juvenile SLE, and pediatric SLE.
• No definitive genetic markers distinguish adult from childhood-onset SLE.

Conclusions:

• The term "childhood-onset SLE" is proposed for individuals diagnosed with SLE before age 18.
• This nomenclature aligns with the definition of childhood and parallels naming conventions for other chronic diseases with early onset.
• Standardizing terminology is crucial for consistent research and clinical management of pediatric SLE.