Immunoglobulin G4-Related Orbital Disease with Bilateral Optic Perineuritis and Maxillary Nerves Involvement: A Case Rep
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CASE REPORT
Immunoglobulin G4-Related Orbital Disease with Bilateral Optic Perineuritis and Maxillary Nerves Involvement: A Case Report Chih-Heng Hung
. Cheng-Yu Lo
Received: August 5, 2020 / Accepted: October 3, 2020 Ó The Author(s) 2020
ABSTRACT Immunoglobulin G4-related optic neuropathy caused by optic perineuritis is a rare complication of immunoglobulin G4-related disease (IgG4-RD). Herein, we report a 38-year-old Asian man with history of sinusitis who presented with painless blurred vision and proptosis for over 6 months. Examination with the Hertel exophthalmometer revealed 21.5 mm on both eyes. Magnetic resonance imaging revealed a doughnut sign encircling the right optic nerve, bilateral tram-track signs on both optic nerves, enlarged bilateral maxillary nerves with perineural spreading to the infraorbital nerves, hypertrophy of extraocular muscles, and pansinusitis. Visual evoked potentials displayed bilateral delayed P100 latency, indicating bilateral optic neuropathy. Biopsy with functional
endoscopic sinus surgery demonstrated diffuse dense lymphoplasmacytic infiltrate and fibrosis. IgG4-positive plasma cells exceeded 50 cells per high-power field while the overall IgG4/IgG ratio was above 40%. Serological studies unveiled extremely high serum concentrations of IgG4 (2650 mg/dL), and the calculated serum IgG4/IgG ratio was 100%. These comprehensive features supported the diagnosis of IgG4-RD with bilateral optic perineuritis, branches of trigeminal nerve involvement, and pansinusitis. The visual acuity improved slightly following the initiation of treatment with corticosteroids, but it became worse again during the tapering course. Following another course of corticosteroids followed by subsequent immunosuppressant treatment with azathioprine, vision in both eyes ultimately improved during the 2-year follow-up period.
Keywords: IgG4-related disease; Maxillary nerve; Optic perineuritis; Orbital disease
C.-H. Hung (&) Department of Ophthalmology, Cathay General Hospital, Taipei, Taiwan e-mail: [email protected] C.-Y. Lo Department of Pathology, Cathay General Hospital, Taipei, Taiwan
Ophthalmol Ther
Key Summary Points IgG4-related optic neuropathy (IgG4RON) caused by optic perineuritis is a rare complication of IgG4-related disease (IgG4-RD). Limited information is available in the literature on the clinical and radiologic risk factors associated with the optic perineuritis type of IgG4-RON. We report a rare case of IgG4-RD with bilateral optic perineuritis, enlarged maxillary nerves with perineural spreading to infraorbital nerves, and pansinusitis; with the diagnosis confirmed by histopathologic study of sinus biopsy specimens. Magnetic resonance imaging is a useful tool for diagnosing cranial nerve perineuritis, with the presence of the ‘‘doughnut’’ sign and ‘‘tram-track’’ sign indicative of inflamed soft tissues surrounding the cranial nerve. The case described here is a ‘‘pure’’ case of optic perineuritis secondary to IgG4-RD, and a successful post-treatment visual outcome was obtained
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