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[IgG4-related ophthalmopathy].
S G Radenska-Lopovok1,2, A S Tertychnyy1, A A Timakova3
1Sechenov First Moscow State Medical University, Moscow, Russia.
Arkhiv Patologii
|July 26, 2022
Summary
Immunoglobulin G4-related disease (IgG4-RD) can manifest as IgG4-related ophthalmopathy, forming lymph node-like structures in the orbit. This rare condition involves not only plasma cells but also CD8+ and CD68+ cells in its unique inflammatory infiltrate.
Area of Science:
- Ophthalmology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition.
- IgG4-RD presents with fibrotic lesions mimicking malignancy and elevated serum IgG4 levels.
- IgG4-related ophthalmopathy is a specific manifestation requiring differential diagnosis from other orbital pathologies.
Purpose of the Study:
- To present clinical cases of IgG4-related ophthalmopathy.
- To investigate the cellular composition and tissue structures in IgG4-related ophthalmopathy.
- To elucidate the pathogenesis and morphogenesis of this rare IgG4-RD manifestation.
Main Methods:
- Clinical case series analysis.
- Histopathological examination of orbital tissues.
- Immunohistochemical staining for CD138, IgG, IgG4, CD8, and CD68.
Main Results:
- Formation of follicle-like structures resembling lymph nodes in orbital tissues.
- Presence of IgG, IgG4, and CD138-positive plasma cells in the inflammatory infiltrate.
- Significant infiltration of CD8+ and CD68+ cells within the lymphohistiocytic infiltrate.
Conclusions:
- IgG4-related ophthalmopathy is a rare manifestation of IgG4-RD.
- Orbital tissue in IgG4-related ophthalmopathy exhibits lymph node-like structures.
- Pathogenesis involves IgG, IgG4, CD138-positive plasma cells, and notably CD8+ and CD68+ cells, highlighting the complexity of the immune response.
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