Childhood-Onset Systemic Lupus Erythematosus Research over the Past Decade in Japan

TL;DR

Research on childhood lupus in Japan has grown since 2015, focusing on kidney disease and immune signaling, with a push for steroid-sparing treatments.

Contribution

A comprehensive review of Japanese cSLE research from 2015 to 2025, highlighting trends and gaps in clinical and translational studies.

Findings

Lupus nephritis and type I interferon signaling are central research themes in Japanese cSLE studies.

Mycophenolate mofetil, tacrolimus, and early belimumab show promise as glucocorticoid-sparing therapies.

Multicenter collaboration is critical for improving pediatric-specific evidence and clinical guidelines.

Abstract

What are the main findings?
Research on childhood-onset SLE in Japan has grown notably since 2015.Lupus nephritis and type I interferon signaling remain central research themes.

Research on childhood-onset SLE in Japan has grown notably since 2015.

Lupus nephritis and type I interferon signaling remain central research themes.

What are the implications of the main findings?
Accumulating evidence supports GC-sparing approaches in childhood-onset SLE.Multicenter collaboration is essential to strengthen pediatric-specific evidence.

Accumulating evidence supports GC-sparing approaches in childhood-onset SLE.

Multicenter collaboration is essential to strengthen pediatric-specific evidence.

Background: Childhood-onset systemic lupus erythematosus (cSLE) is a rare, serious autoimmune disease characterized by multiorgan involvement and long-term morbidity. Although several studies have examined this condition in Japan, a comprehensive summary of recent findings remains lacking. Methods: PubMed was searched for Japanese publications on cSLE published between 2015 and 2025, including clinical studies, case reports, translational research, basic science studies, systematic reviews, clinical practice guidance, and transition care guidance. Results: Sixty publications met the inclusion criteria: 20 clinical studies, 30 case reports, 6 translational studies, 1 basic science study, 1 systematic review, 1 clinical practice guidance, and 1 transition care guidance. Most clinical studies were retrospective, although multicenter and registry-based designs have increased in recent years. Lupus nephritis remained the primary research focus, with accumulating evidence supporting mycophenolate mofetil, tacrolimus, and early belimumab as glucocorticoid (GC)-sparing approaches. Case reports illustrated the broad clinical spectrum of cSLE, with hematological and vascular complications being the most frequently reported. Translational studies highlighted the pathogenic role of type I interferon signaling and cytokine dysregulation, particularly in macrophage activation syndrome. Despite these advances, prospective studies and standardized assessment methods for pediatric-specific practice remain limited. Conclusions: Over the past decade, cSLE research in Japan has contributed to a deeper understanding of its clinical and immunological characteristics. However, treatment-related complications and long-term organ damage remain important challenges. Continued multicenter collaboration and domestic data accumulation may strengthen the evidence base, facilitate optimization of GC-sparing approaches, improve clinical management, and provide background information to support future discussions on clinical practice guidelines.

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