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IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland

Norberto Mancera1, Jasmina Bajric1, Curtis E Margo1,2

  • 1Department of Ophthalmology, University of South Florida , Tampa, Florida, USA.

Orbit (Amsterdam, Netherlands)
|November 15, 2019
PubMed

Insights

A rare case of IgG4-rich reactive lymphoid hyperplasia in the lacrimal gland was identified in a young woman. Its relationship to IgG4-related disease and lymphoma requires further investigation.

Area of Science:

  • Ophthalmology
  • Immunology
  • Pathology

Background:

  • Immunoglobulin G4 (IgG4)-related disease is a fibroinflammatory condition affecting various organs.
  • Lacrimal gland involvement can occur in IgG4-related disease, but reactive lymphoid hyperplasia is not a typical presentation.

Observation:

  • A 24-year-old woman presented with reactive lymphoid hyperplasia of the lacrimal gland.
  • Histopathology revealed a high density of IgG4-positive plasma cells (40-60% of IgG+ cells).
  • Modest elevation in serum IgG4 levels was noted.

Findings:

  • The patient showed a transient response to corticosteroids, followed by treatment with rituximab.
  • This condition is not currently recognized as part of the IgG4-related disease spectrum or a lymphoma precursor.
  • The biological potential and long-term behavior of IgG4-rich reactive lymphoid hyperplasia are unknown.

Implications:

  • This case highlights a distinct entity of IgG4-rich reactive lymphoid hyperplasia in the lacrimal gland.
  • Further research is needed to determine the natural history and potential for progression to IgG4-related disease or lymphoma.
  • Understanding this condition may refine diagnostic criteria and treatment strategies for IgG4-related orbital conditions.

Keywords:
DacryoadenitisIgG4lacrimal glandlymphoid hyperplasialymphoma

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