Radiological Determinants of Complicated Immunoglobulin G4–Related Ophthalmic Disease: A Territory-Wide Cohort Study

Abstract

Purpose: To evaluate the presenting radiological features of immunoglobulin G4–related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). Methods: A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. Results: A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. Conclusions: In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.

Corneal endothelial evaluations in immunoglobulin g4‐related ophthalmic disease

“…4 Orbital involvement had been found in up to 95% of IgG4-ROD patients. 1 In this study, we hypothesize that there are changes in the corneal endothelium in IgG4-ROD patients.…”

“…In their recent editorial, the new Editors of the journal were keen to confirm the rules for the use of the term data, insisting that it is a plural noun. 1 In support of the Editors, the Oxford English Dictionary (OED)-nominally the gold standard for the English language-advises that the use of data as a singular is, to use the inimitable OED style, 'catachrestic' (i.e., wrong). One might note, however, that in the same edition the OED expends, for example, nearly 30 column centimetres-and nine prior definitions-before allowing that the word gay may mean homosexual.…”

“…Corneal endothelial evaluations in immunoglobulin g4-related ophthalmic disease Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) was first found in patients with sclerosing dacryoadenitis and subsequently reported in other parts of the ocular adnexa. 1 Ocular surface changes and visualthreatening complications including optic neuropathy and malignant changes were reported. 2,3 The endothelium is the innermost layer of the cornea that separates the corneal stroma from the aqueous humour, and its function is crucial to maintaining corneal transparency.…”

“…We graded the extent of ocular involvement on a scale from 0 to 5 by counting the presence of the following seven involved items radiologically: eyelid, lacrimal gland, orbital mass, 1-2 extraocular muscles, 3 or more extraocular muscle, frontal nerve and infraorbital nerve. 1 The degree of meibomian gland dropout was measured using the ImageJ software by dividing the meibomian gland dropout area by the total area of the tarsal conjunctiva. 3 The comparison of corneal endothelium parameters between IgG4-ROD patients and healthy subjects was measured using the independent samples t-test or Mann-Whitney tests.…”