An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD. Issue 3 (2nd September 2014)

• Record Type: Journal Article
• Authors: Andrew, Nicholas H; Sladden, Nicole; Kearney, Daniel J; Selva, Dinesh
• Is Part Of: British journal of ophthalmology. Volume 99:Issue 3(2015)
• Page Start: 376
• Page End: 381
• Publication Date: 2014-09-02
• Subjects: Orbit -- Pathology -- Lacrimal gland -- Inflammation; Ophthalmology -- Periodicals; 617.7
• DOI: 10.1136/bjophthalmol-2014-305545
• Languages: English
• ISSNs: 0007-1161

Abstract

Abstract : Aim: To determine the proportion of idiopathic orbital inflammation (IOI) and orbital benign lymphoid hyperplasia (OBLH) accounted for by immunoglobulin (Ig)G4-related orbital disease (IgG4-ROD) using the comprehensive diagnostic criteria for IgG4-related disease published by Umehara et al and the consensus diagnostic criteria published by Deshpande et al . Secondary aims were to compare the histological and clinical features of IgG4-ROD and non-IgG4-ROD cases, and to compare IgG4-ROD cases diagnosed using the comprehensive diagnostic criteria with those diagnosed using the consensus diagnostic criteria. Methods: A retrospective histopathological review and clinical case series. 55 cases of biopsy-confirmed non-granulomatous IOI and 10 cases of biopsy-confirmed OBLH were included. The intensity of sclerosis, lymphoplasmacytic infiltration and eosinophilic infiltration was graded from 0 to 3+ using a standardised and validated scoring system. Results: IgG4-ROD accounted for 50% and 40% of cases originally diagnosed as OBLH and 23.6% and 5.4% of cases originally diagnosed as IOI, using the comprehensive diagnostic criteria and the consensus diagnostic criteria, respectively. IgG4-ROD cases had numerous significant histological differences, but relatively few significant clinical differences, from non-IgG4-ROD cases. Compared with the comprehensive diagnostic criteria, the consensus diagnostic criteria identified a group of IgG4-ROD cases with a slightly higher ratio of IgG4+ to IgG+ (p=0.01) and a slightly longer duration of symptoms (p=0.02). Conclusions: This is the largest review of IgG4 staining among biopsy-confirmed IOI and OBLH. IgG4-ROD accounted for a substantial proportion of OBLH. The prevalence among cases of IOI was considerably reduced when the consensus diagnostic criteria were used in place of the comprehensive diagnostic criteria.