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.
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
