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
Clinical Scorecard: Pediatric IgG4-Related Disease: A Case Series Highlighting Clinical Variability and Management Challenges
At a Glance
Category Detail
Condition IgG4-related disease (IgG4-RD) Key Mechanisms Chronic immune-mediated fibro-inflammatory condition characterized by IgG4-expressing plasma cells and associated histopathological features. Target Population Pediatric patients (ages 4–17 years) Care Setting Single-center observational study
Key Highlights
Clinical manifestations include orbital pseudotumor, diabetes insipidus, and systemic inflammatory signs. Diagnosis often delayed by 3 to 12 months due to symptom mimicry. Treatment regimens varied, including glucocorticoids and cytostatic agents. Three patients achieved good clinical and radiological response; others experienced relapses. Histopathological confirmation required for diagnosis, focusing on lymphoplasmacytic infiltration and fibrosis. Guideline-Based Recommendations
Diagnosis
Utilize the 2020 Revised Comprehensive Diagnostic criteria for IgG4-RD. Confirm diagnosis through biopsy showing lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis. Management
Initial treatment may include glucocorticoids combined with cytostatic agents or monotherapy. Consider rituximab therapy for incomplete remission. Monitoring & Follow-up
Regular follow-up to assess clinical response and monitor for relapses. Risks
Potential for misdiagnosis leading to delays in appropriate treatment. Risk of relapses during treatment, necessitating adjustments in therapy. Patient & Prescribing Data
Six pediatric patients with confirmed IgG4-RD.
Effective treatments included glucocorticoids and cytostatic agents, with some patients requiring systemic therapy after relapses.
Clinical Best Practices
Early recognition of symptoms to facilitate timely diagnosis. Utilization of comprehensive diagnostic criteria to avoid misdiagnosis. Tailoring treatment regimens based on individual patient response and disease severity. Related Resources & Content
