IgG4-related disease in patients with idiopathic orbital inflammation

Abstract

Background To identify the prevalence of positive IgG4 immunostaining in orbital tissue among patients previously diagnosed with nongranulomatous idiopathic orbital inflammation (IOI) and to compare the clinical characteristics of patients with and without IgG4-positive cells. Methods A retrospective review of all patients with a histopathologic diagnosis of IOI was performed. Immunohistochemical staining was performed to identify IgG-positive cells and IgG4-positive cells. Multivariate analysis was performed using likelihood ratio-test logistic regression on the differences between IgG4-related disease (IgG4-RD) and non-IgG4-RD. Results Of the 45 patients included, 21 patients (46.7%) had IgG4-positive cells, with 52.4% being male and a mean age of 55.9 ± 13.4 years. Bilateral ocular adnexal involvement (adjusted odds ratio [aOR] = 9.45; P = 0.016) and infraorbital nerve enlargement (aOR = 12.11; P = 0.008) were frequently found in IgG4-RD patients. Complete remission occurred in 23.8% of IgG4-RD patients and 41.7% of non-IgG4-RD patients. IgG4-RD patients had more frequent recurrent disease than non-IgG4-RD patients. Conclusions Nearly 50% of IgG4-RD patients were previously diagnosed with biopsy-proven IOI. IgG4-RD was more frequent in patients with bilateral disease and infraorbital nerve enlargement, showing the importance of tissue biopsy in these patients. Immunohistochemistry studies of all histopathology slides showing nongranulomatous IOI are highly recommended to evaluate for IgG4-RD.

Tolosa–Hunt Syndrome and IgG4 Diseases in Neuro-Ophthalmology

“…The usual recommended initial dose is 1 mg/kg/day, although higher doses are used for patients with intractable disease in the form of optic nerve involvement or active orbital myositis or those with evidence of systemic disease. [ 38 ] A dose less than 10 mg is ineffective for maintaining remission[ 39 ] [ Figure 7 ].…”

A Case of Immunoglobulin G4-Related Ophthalmic Disease With Unilateral Visual Field Impairment

“…The ophthalmologic clinical hallmark of IgG4-related ocular disease is bilateral lacrimal gland enlargement with three features: suborbital nerve enlargement [7], exophthalmos, and compressive optic neuropathy [4]. IgG4-related optic neuropathy is commonly caused by compression of the optic nerve by enlargement of the lacrimal glands or other intraorbital tissues [4,[8][9][10][11].…”

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors

“…A review of 55 previously diagnosed cases of OP demonstrated that over 40% were histologically re‐classified as IgG4‐ROD. In another study, which evaluated cases of previously diagnosed OP, the radiologic findings of bilateral orbital involvement and infraorbital nerve involvement are associated with IgG4‐ROD when tissue samples are assessed by IgG4‐ROD criteria 957,958 …”