An analysis of IgG4‐related ocular disease among idiopathic orbital inflammations and mucosa‐ associated lymphoid tissue lymphoma

Abstract

PurposeTo determine the proportion of idiopathic orbital inflammation (IOI) and mucosa‐associated lymphoid tissue (MALT) lymphoma accounted for by immunoglobulin (Ig) G4‐related orbital disease (ROD) using domprehensive criteria and To report the clinicopathological characteristics of patients with ocular MALT lymphoma with IgG4‐positive ells.MethodsA retrospective histopathological review and clinical case series. 49 patients from January 2005 through August 2015 were included. 20 cases of biopsy‐confirmed IOI, 29 cases of biopsy‐confirmed MALT lymphoma. Immunohistochemistry with IgG and IgG4 immunostaing. MALT lymphoma with IgG4 positive plasma cells were included when the IgG4/IgG ratio was 40% and IgG4 count was over 10/HPF. Histopathologic features, demographic and clinical data, radiologic findings, treatment and follow‐up information for each patient were analysed.ResultsPossible IgG4‐ROD accounted for 50.0% of cases originally diagnosed as IOI and 41.38% of cases originally diagnosed as MALT lymphoma. IgG4 positive MALT lymphoma group has contralateral IgG4 positive chronic inflammation lesion (p = 0.007). Relapse rate is higher than IgG4‐ unrelated group (p = 0.05) but not significantly.ConclusionsOf the MALT lymphoma cases, 12 cases were diagnosed as having possible IgG4‐ROD. Our result have demonstrated that ocular IgG4‐ROD can predispose to the development of ocular adnexal MALT lymphoma. However, further longitudinal observations during the course of disease are needed to confirm whether B‐cell lymphoma originate from IgG4‐ROD.