Clinical Report: Pediatric IgG4-Related Disease Case Series
Overview
This report presents a case series of six pediatric patients with IgG4-related disease (IgG4-RD), highlighting the clinical variability and management challenges associated with this rare condition. The findings emphasize the importance of timely diagnosis and effective treatment strategies.
Background
IgG4-RD is a rare fibro-inflammatory condition that can significantly affect children, yet its clinical spectrum and management in this population are not well understood. The disease can mimic other conditions, leading to diagnostic delays that can complicate treatment. Understanding the pediatric manifestations of IgG4-RD is crucial for improving outcomes and guiding management.
Data Highlights
Patient
Age at Onset
Clinical Manifestation
Treatment Regimen
Response
1
12
Orbital pseudotumor
Glucocorticoids + Cytostatic
Good
2
10
Orbital pseudotumor
Cytostatic monotherapy
Good
3
15
Orbital pseudotumor
Glucocorticoid monotherapy
Good
4
17
Diabetes insipidus
Glucocorticoids + Cytostatic
Incomplete remission
5
14
Fever
Cytostatic monotherapy
Incomplete remission
6
4
Orbital pseudotumor
Glucocorticoids + Cytostatic
Relapse controlled
Key Findings
All six patients met the 2020 Revised Comprehensive Diagnostic criteria for IgG4-RD.
The median age at disease onset was 12 years, with a range from 4 to 17 years.
Orbital involvement was the most common clinical manifestation, seen in four patients.
Treatment regimens varied, including glucocorticoids combined with cytostatics and monotherapy.
Three patients achieved good clinical and radiological responses, while two had incomplete remission.
Diagnostic delays ranged from 3 to 12 months, highlighting the challenges in recognizing pediatric IgG4-RD.
Clinical Implications
Timely recognition and diagnosis of IgG4-RD in children are essential to prevent complications and optimize treatment outcomes. Clinicians should consider a broad differential diagnosis when encountering pediatric patients with atypical presentations, particularly those with orbital involvement. Effective management often requires a combination of glucocorticoids and cytostatic agents.
Conclusion
This case series underscores the clinical variability and management challenges of IgG4-RD in the pediatric population. Enhanced awareness and understanding of this condition are crucial for improving diagnosis and treatment strategies.
by Olga Viktorovna Shpitonkova, Natalia Anatolievna Geppe, Vera Alekseevna Podzolkova, Elena Yurievna Afonina, Tatiana Vladimirovna Zubareva, Maria Nikolaevna Nikolaeva, Natalia Yurievna Golovanova, Maria Kirillovna Osminina, Elena Vasilievna Frolkova, Maria Alekseevna Kudryashova, Nadezhda Stepanovna Podchernyaeva
