Case report: The masquerading spectrum: a pediatric case series of IgG4-related disease - Summary - MDSpire

Case report: The masquerading spectrum: a pediatric case series of IgG4-related disease

  • 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

  • May 8, 2026

  • 0 min

Share

Objective:

To enhance recognition and inform management of pediatric IgG4-related disease (IgG4-RD) through a case series of six children, focusing on clinical implications.

Key Findings:
  • The most common clinical manifestation was orbital involvement, with four patients presenting with orbital pseudotumor.
  • Diagnostic delays ranged from 3 to 12 months.
  • Three patients achieved good clinical and radiological responses, while one experienced multiple relapses managed by switching therapies, and two achieved incomplete remission.
Interpretation:

The study highlights the diagnostic challenges and phenotypic diversity of pediatric IgG4-RD, emphasizing the need for increased awareness and tailored management strategies to improve patient outcomes.

Limitations:
  • Small sample size limits generalizability.
  • Lack of long-term follow-up data for all patients.
  • Potential biases or confounding factors affecting the study's conclusions.
Conclusion:

Pediatric IgG4-RD presents with diverse clinical manifestations and requires careful diagnosis and management, with glucocorticoids and cytostatic agents proving effective in many cases.

Original Source(s)

Related Content