Abstract

BackgroundThe following study investigates the involvement of optic neuropathy in IgG4-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4-ROD related optic neuropathy related to other orbital diseases.MethodsThis retrospective study included 225 patients with IgG4-RD admitted at two ophthalmology centers between January 2014 and December 2017. Twenty-six patients had both pre-therapeutic orbital MRI and optic never injury. The causes of optic neuropathy were analyzed, and the special sign in MRI to diagnose IgG4-ROD was also evaluated.ResultsTwelve cases had inflammation of the optic nerve sheath, while 14 cases had compression due to extraocular muscles and pseudo tumor masses. Two cases had hypertrophic cranial pachymeningitis, while one case had hypophysis involving optic chiasma.ConclusionThe most common causes of optic nerve injury in IgG-4 ROD are inflammation of optic nerve sheath, compression of extraocular muscles, pseudo tumor mass and hypertrophic cranial pachymeningitis, and hypophysis involving optic chiasma.

Highlights

  • The following study investigates the involvement of optic neuropathy in Immunoglobulin G4 (IgG4)-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4ROD related optic neuropathy related to other orbital diseases

  • We suggest a radiological diagnostic criterion for optic neuropathy cases diagnosed as IgG4-ROD

  • The exclusion criteria were: ocular manifestations were not the first symptoms; pretherapeutic MRI was insufficient for an adequate interpretation; absence of IgG4 testing during the pathology examination; thyroid-related eye diseases and lymphoma and other orbital- related diseases; ocular exam that was insufficient for analysis; patients without visual loss or optic neuropathy

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Summary

Introduction

The following study investigates the involvement of optic neuropathy in IgG4-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4ROD related optic neuropathy related to other orbital diseases. Target organs that are most commonly affected by IgG4-RD are the orbits, salivary and lacrimal glands, lungs, kidneys, prostate, aorta and retroperitoneum, lymph nodes, biliary tree and as visceral inflammatory pseudo tumours. In the USA, 23% of all of IgG4RD cases, the disease manifests in the eye or orbit. A number of orbital inflammatory cases, Optic neuropathy may be involved in IgG4-ROD, and it can lead to severe injuries. This type of disease is more likely misdiagnosed as other orbital inflammatory diseases, such as optic neuritis, Graves’ disease, inflammatory pseudotumor, lymphoma, sarcoidosis and so on [6]. Much knowledge has been gained about the clinical manifestations of IgG4-ROD [7]; yet, most of studies were small case

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