IgG4-Related Disease Presenting as Isolated Scleritis
Eran Berkowitz1, Ella Arnon1, Alona Yaakobi1
1Department of Ophthalmology, Hillel Yaffe Medical Center Affiliated with The Bruce Rappaport School of Medicine, The Technion, Haifa, Israel.
Case Reports in Ophthalmological Medicine
|February 3, 2017
Summary
A rare case of IgG4-related disease (IgG4-RD) presenting as nodular scleritis in a young woman was successfully treated with corticosteroids and methotrexate. This highlights IgG4-RD as a crucial differential diagnosis for scleritis.
Area of Science:
- Ophthalmology
- Rheumatology
- Immunology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
- Scleritis, inflammation of the sclera, can be associated with systemic autoimmune diseases.
Observation:
- A 20-year-old female presented with acute nodular scleritis of the left eye.
- Initial systemic corticosteroid treatment provided limited improvement.
Findings:
- Ocular biopsy revealed histopathological features consistent with IgG4-related disease.
- Long-term therapy with corticosteroids and methotrexate achieved significant symptom resolution.
Implications:
- IgG4-related disease should be considered in the differential diagnosis of refractory scleritis, especially in young adults.
- Further research may explore organ-specific pathophysiological variations in IgG4-RD, potentially involving T cell mechanisms.
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