A clinicopathological study of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders

Abstract

To study the clinicopathologic features of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders. Forty-three cases were selected from the Department of Pathology, West China Hospital, Sichuan University from December 2008 to January 2015 with retrospective series case study. The clinical manifestation, laboratory examination, imaging findings, treatment and histopathological characteristics were analyzed. According to the diagnostic criteria for IgG4-related ocular disease in 2014 and relevant literatures, our cases were divided into two groups: IgG4-related ocular disease (23 samples from 18patients) and non-IgG4-related ocular disease (31 samples from 25 patients). The male-to-female ratio, the periductal fibrosis and the orbital lesion area was analyzed by Pearson chi-square. The Immunohistochemistry of IgG4+/HPF, IgG+/HPF and IgG4+/IgG+ ratio and age was analyzed by two sample t test. The involved structural tissue and the infiltration of eosinophils and lymphocytes and plasma cells was analyzed by signed-rank test. The significant statistic differences were observed between these two groups in immunohistochemical staining of IgG4 (>40/HPF) (t=12.17, P<0.01), IgG (t=2.91, P<0.01) and IgG4+/IgG+ ratio (≥40%) (t=16.98, P<0.01), the lymphocytic and plasmacytic infiltration (F=3.62, P=0.03),the periductal fibrosis(F=4.71 , P=0.03), the involved region of orbit (t=1.11, P=0.01), but not in the mean age and male-to-female ratio (t=1.09, P=0.58). IgG4 ROD should be distinguished form the non-specific inflammation disorders, lymphoma and so on. IgG4-related ocular disease often arises from middle-old man, characterized by bilateral orbital painless swelling and abundant IgG4+ plasma cells infiltration. Nearly all orbital tissue could be involved by IgG4-related ocular disease, especially lacrimal gland. But the characteristic obliterating phlebitis were not seen. The immunohistochemical staining of IgG4 and IgG plays an important role in differentiating IgG4-related ocular disease from non-IgG4-related ocular disorders. Combining clinical manifestation, laboratory examination, imaging findings with histopathology is helpful for diagnosis and differential diagnosis. (Chin J Ophthalmol, 2016, 52: 256-262).