Abstract
PurposeTo investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD).MethodsA total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD.ResultsMale predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland.ConclusionsLacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.
Highlights
IgG4-related disease (IgG4-RD) is a recently recognized systemic fibro-inflammatory condition, histologically characterized by numerous lymphoplasmacytic infiltrations with a predominance of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis in tumourlike lesions of multiple organs [1], usually accompanied with elevated serum IgG4 levels
Patients with the enlargements of extraocular muscles and trigeminal nerve exhibit a trend to have a higher risk for relapse as well [3]
A total of 573 IgG4-RD patients were enrolled in the cohort at Peking Union Medical College Hospital from 2011 to January 2020 (ClinicalTrials.gov ID: NCT01 670695)
Summary
IgG4-related disease (IgG4-RD) is a recently recognized systemic fibro-inflammatory condition, histologically characterized by numerous lymphoplasmacytic infiltrations with a predominance of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis in tumourlike lesions of multiple organs [1], usually accompanied with elevated serum IgG4 levels. Gan et al BMC Ophthalmology (2021) 21:447 including lacrimal gland, extraocular muscles, trigeminal nerve and orbital fat, are frequently affected by IgG4-RD [2, 3], forming the conception of IgG4-related ophthalmic disease (IgG4-ROD). It was initially defined as different entities, such as Mikulicz’s disease, idiopathic orbital inflammation or orbital benign lymphoid hyperplasia before the recognition of IgG4-RD [2]. Lacrimal gland involvement is the most common ophthalmic manifestation of IgG4-RD and is frequently associated with extra-orbital diseases [5]. Patients with the enlargements of extraocular muscles and trigeminal nerve exhibit a trend to have a higher risk for relapse as well [3]
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