# Anterior scleritis with IgG4 lymphoplasmacytic infiltration: a case report

**Authors:** Poojitha Balakrishnan, Matthew G. Vicinanzo, John P. Luckett, Tom Winokur, Ryan S. Weldon, Russell W. Read

PMC · DOI: 10.1186/s12348-025-00516-3 · Journal of Ophthalmic Inflammation and Infection · 2025-08-16

## TL;DR

A rare case of eye inflammation was linked to IgG4-related disease, highlighting its importance in diagnosing similar conditions.

## Contribution

This case report presents a rare isolated presentation of IgG4-related disease as nodular anterior scleritis.

## Key findings

- The patient had chronic unilateral nodular anterior scleritis with partial response to treatment.
- Histopathology confirmed IgG4 lymphoplasmacytic infiltration in the scleral tissue.
- Scleritis can be a rare initial sign of IgG4-related disease.

## Abstract

To report a case of chronic unilateral nodular anterior scleritis as a rare, isolated presentation of IgG4-related ophthalmic disease.

A fifty-two-year-old patient was evaluated for painful, nodular scleral injection of the right eye, diagnosed as nodular anterior scleritis. There was only a partial response to topical corticosteroid and systemic immunomodulatory therapy. This, in combination with a sharp delineation between normal and abnormal sclera lead to the decision to perform a diagnostic biopsy of the lesion. Histopathology showed an IgG4 lymphoplasmacytic infiltration of the scleral tissue.

There are few documented reports of scleritis as the presenting manifestation of IgG4-related disease. IgG4-related disease is an increasingly recognized etiology to be considered in evaluating and managing scleritis.

## Linked entities

- **Diseases:** IgG4-related disease (MONDO:0017287), scleritis (MONDO:0001718)

## Full-text entities

- **Genes:** SDC1 (syndecan 1) [NCBI Gene 6382] {aka CD138, SDC, SYND1, syndecan}
- **Diseases:** Ophthalmic Disease (MESH:C535922), rheumatoid arthritis (MESH:D001172), IgG4 (MESH:D000077733), fibrosis (MESH:D005355), ulcerative colitis (MESH:D003093), diabetes mellitus (MESH:D003920), retroperitoneal or aortic fibrosis (MESH:D012185), obliterative phlebitis (MESH:D010689), Anterior scleritis (MESH:D015423), inflammation (MESH:D007249), hypertension (MESH:D006973), pain (MESH:D010146), pancreatitis (MESH:D010195), photophobia (MESH:D020795)
- **Chemicals:** Hematoxylin (MESH:D006416), timolol (MESH:D013999), H&amp;E (MESH:D006371), steroids (MESH:D013256), eosin (MESH:D004801), difluprednate (MESH:C015808), rituximab (MESH:D000069283), prednisone (MESH:D011241), adalimumab (MESH:D000068879), dorzolamide (MESH:C062765)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

2 references — full list in the complete paper: https://tomesphere.com/paper/PMC12357819/full.md

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Source: https://tomesphere.com/paper/PMC12357819