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Clinicopathological features differentiating Castleman Disease from IgG4-related ophthalmic disease, a 10 years
Hetong Wang1, Jinzhi Zhao, Wei Wang
1Tianjin Key Laboratory of Retinal Functions and Diseases, Tianjin Branch of National Clinical Research Center for Ocular Disease, Eye Institute and School of Optometry, Tianjin Medical University Eye Hospital, Tianjin, China.
Purpose:
This study compared two rare diseases that present with enlarged lacrimal glands and analyzed their clinical features, among others, for identification.
Methods:
Setting - A single-center retrospective study.
Patient Or Study Population:
Patients treated at our center from 2013 to 2023 with pathologically diagnosed IgG4-ROD (n = 31, 42 eyes) and CD (n = 5, 5 eyes).
Intervention Or Observation Procedures:
No interventions administered.
Main Outcome Measures:
The analysis included patient demographics, laboratory tests, histopathological tests, imaging studies, treatment, and outcomes. Data collection involved visualizing medical records.
Results:
The lacrimal glands/eyelid soft tissues and extraocular muscles of the IgG4-ROD group were affected. Histopathological examination showed lymphoid proliferation in both groups had lymphocyte hyperplasia, and the pathological IgG4+/IgG+ ratio in the IgG4-ROD group was higher than that in the CD group (P = 0.0057), which was more than 40%. IgG4-ROD exhibited significantly higher MASSON positivity than CD (78.1% vs. 33.3%, P = 0.0466). CD lesions exhibited a 50% reduction in Ki-67 proliferative activity relative to IgG4-ROD (P = 0.0349). Bone destruction was identified in 20% of CD versus none in IgG4-ROD (P = 0.024). Conversely, fibrotic imaging features demonstrated a significantly higher prevalence in IgG4-ROD (40%) compared to CD (P = 0.0103).
Conclusions:
Significant differences in pathology and imaging exist between IgG4-ROD and CD. IgG4-ROD is characterized by an IgG4+/IgG+ plasma cell ratio exceeding 40% and extensive fibrosis. Imaging predominantly reveals nondestructive fibrotic foci, reflecting the chronic inflammatory fibroproliferative nature of the disease. Conversely, CD exhibits higher local invasiveness and lower cellular proliferative activity.
