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IgG4 disease.

Motohisa Yamamoto¹, Masato Hashimoto, Hiroki Takahashi

¹Department of Gastroenterology, Rheumatology and Clinical Immunology (MY, HT, YS), Sapporo Medical University School of Medicine, Sapporo, Japan; and Department of Ophthalmology (HM), Sapporo Medical University School of Medicine, Sapporo, Japan.

Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society

|November 19, 2014

Summary

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition affecting multiple organs. This review covers IgG4-RD diagnosis, treatment with corticosteroids, and potential relapses.

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Area of Science:

Rheumatology
Ophthalmology
Pathology

Background:

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition.
Characterized by elevated IgG4 serum levels and IgG4-positive plasma cell infiltration.
Affects organs like lacrimal/salivary glands, pancreas, thyroid, lungs, and kidneys.

Observation:

IgG4-related ophthalmic disease involves orbital myositis, perineuritis, and inflammation.
Infraorbital nerve enlargement on MRI is a key diagnostic sign.
Systemic screening is crucial for diagnosis and differentiating from neoplasms.

Findings:

Corticosteroids are effective for inducing remission in IgG4-RD.
Relapses can occur during corticosteroid tapering.
This review details the history, clinical features, diagnostics, treatment, and prognosis.

Implications:

Accurate diagnosis of IgG4-RD requires systemic evaluation.
Understanding disease progression and relapse patterns is vital for patient management.
Further research may improve long-term treatment strategies for IgG4-RD.