Immunoglobulin G4 (IgG4)-Positive or -Negative Ocular Adnexal Benign Lymphoid Lesions in Relation to Systemic Involvement

Abstract

The purpose of this study is to determine the relationship of ocular adnexal benign or reactive lymphoid hyperplasia, including orbital pseudotumor, with immunoglobulin G4 (IgG4)-related diseases. Medical charts of 9 consecutive patients with ocular adnexal benign lymphoid lesions, seen in the Department of Ophthalmology, Okayama University Hospital, were reviewed, and pathological sections were restained immunohistochemically for IgG4-, IgG-, and CD138-positive plasma cells. The diagnosis of IgG4-positive lesions was based on 10 or more IgG4-positive plasma cells in a high-power field and greater than 40% ratios of IgG4-positive plasma cells/CD138-positive plasma cells and IgG4-positive plasma cells/IgG-positive plasma cells. IgG4-positive lesions were determined as absent in 5 patients (4 with bilateral lacrimal/orbital lesions and one with a unilateral conjunctival lesion), none of whom showed systemic manifestations. In contrast, IgG4-positive lesions were present in 4 patients (3 with bilateral lacrimal/orbital lesions and one with a unilateral lacrimal/orbital lesion), who showed systemic manifestations : one with Hashimoto thyroiditis, one with IgG4-positive bilateral interstitial lung disease and hepatic inflammatory pseudotumor, one with bilateral interstitial lung disease, and one with systemic lymphadenopathy and antiphospholipid syndrome. In conclusion, IgG4-positive ocular adnexal benign lymphoid lesions might be used as a benchmark for the probable presence of other systemic lymphoid lesions.

Bilateral Conjunctival Lesions in Blastic Plasmacytoid Dendritic Cell Neoplasm

“…Tissue sections were processed by standardized heating pretreatment (heating at 100°C for 30 min) for antigen retrieval prior to entering the immunohistochemical procedures. 10 In brief, the sections were incubated with 3% hydrogen peroxide for 5 min to inactivate endogenous peroxidase and blocked with 10% normal goat serum for 10 min. The sections were then incubated with primary antibodies overnight at 4°C, washed with 0.05% Tween 20-containing phosphate buffered saline three times, incubated with the secondary antibody at room temperature for 30-60 min, and washed.…”

“…[8][9][10] Leukemic infiltration of the conjunctiva is rare but does occur to present the same clinical picture as lymphoma. [11][12][13][14][15] In the older literature, three patients have been described in separate case reports to develop multiple skin lesions and a conjunctival lesion in the course of acute myelomonocytic leukemia.…”

“…5 Other less common types of primary lymphoma in the conjunctiva, which have been reported so far, include T-cell lymphoma 6 and Burkitt lymphoma. 7 Benign lymphoid hyperplasia [8][9][10] and leukemic infiltration [11][12][13][14][15] also manifest as conjunctival masses. All conjunctival lesions, including lymphoma, leukemic infiltration, and benign lymphoid hyperplasia, present the same clinical feature called salmon-pink lesions.…”

Systemic immunoglobulin G4 (IgG4) disease and idiopathic orbital inflammation; removing ‘idiopathic’ from the nomenclature?

“…[22][23][24][25][26][27][28] IgG4-associated disease is now recognised as a systemic disease process. 22 Table 2 describes conditions once viewed as unique but which are now recognised by the IgG4-related systemic disease spectrum.…”

Rituximab for the treatment of IgG4-related orbital disease: experience from five cases

“…[1][2][3][4] Retrospective IgG4 staining of orbital biopsies has revealed that IgG4-RD may account for 36% of cases originally diagnosed as idiopathic orbital inflammation, 5 and an even higher proportion of orbital lymphoid hyperplasia. 6,7 Corticosteroids are considered first-line treatment for IgG4-ROD and the response is typically excellent but unsustained. A metaanalysis of published cases of IgG4-related orbital disease (IgG4-ROD) revealed that 50% of all cases treated with corticosteroids experienced disease relapse during dose taper or shortly after corticosteroid cessation.…”