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IgG4-related disease presenting as panuveitis without scleral involvement
Kinda Najem1, Larissa Derzko-Dzulynsky2, Edward A Margolin2,3
1Department of Ophthalmology, Université de Montréal, 5415 de l'Assomption Boulevard, Montreal, Quebec, H1T 2M4, Canada. kinda.najem@gmail.com.
Journal of Ophthalmic Inflammation and Infection
|March 1, 2017
Summary
This case highlights IgG4-related disease (RD) in diagnosing intraocular inflammation and cranial nerve palsies. It presents a unique case of IgG4-RD associated panuveitis without scleral involvement.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Intraocular inflammation and cranial nerve palsies can present complex diagnostic challenges.
- Immunoglobulin G4-related disease (IgG4-RD) is an emerging condition that requires consideration in the differential diagnosis of such cases.
Purpose of the Study:
- To report a unique case of IgG4-related disease (RD) presenting with intraocular inflammation and multiple cranial nerve palsies.
- To emphasize the importance of including IgG4-RD in the differential diagnosis of similar clinical presentations.
Main Methods:
- A case study of a 33-year-old man with a history of idiopathic bilateral panuveitis.
- Clinical examination, magnetic resonance imaging/angiography (MRI/MRA), and histopathological biopsy of a skull base lesion.
Main Results:
- The patient experienced recurrent cranial nerve palsies (third and sixth nerve) and decreased visual acuity.
- MRI/MRA revealed an osteodestructive skull base process involving the cavernous sinus and superior orbital fissure.
- Biopsy confirmed the diagnosis of IgG4-related disease (RD).
Conclusions:
- This is the first reported case of IgG4-related disease (RD) associated panuveitis without scleral involvement.
- The findings expand the spectrum of clinical manifestations associated with IgG4-RD.
- Early recognition and diagnosis of IgG4-RD are crucial for appropriate management.
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