IgG4-related disease presenting as posterior scleritis and vitritis, progressing to multifocal orbital involvement

Abstract

IgG4-related disease (IgG4-RD) is a rare, chronic inflammatory condition that may involve nearly every organ system. Originally identified as a cause of autoimmune pancreatitis, its characteristic histological and clinical features have been found in a wide variety of inflammatory presentations, including the eye and orbit. Here we describe an example of a case of IgG4-RD initially presenting as scleritis and vitritis, with further progression to multifocal bilateral orbital involvement. Tissue biopsy of an orbital mass was highly characteristic of IgG4-RD histology and a rapid clinical response to corticosteroids was observed. This case highlights IgG4-RD as a rare cause of intraocular inflammation that may progress to involve the orbit.

Keywords: Immunology; Ophthalmology; Retina; Rheumatology.

Figure 1

Images of ocular and orbital IgG4 diseases. (A) and (B) Optomap and optical coherence tomography images demonstrating initial serous retinal detachment. (C) T2-weighted image demonstrating hypointense scleral thickening on initial presentation (arrow). (D) T2 image demonstrating progression, with multifocal hypointense lesions within the left globe and adjacent to the right lamina papyracea (arrows).

Figure 2

Histopathology of IgG4-related orbital disease. (A) H&E, x 40 and (B) H&E, x 400. The orbital fibrofatty tissue is extensively infiltrated by inflammatory cells, which consist of scattered aggregates of mixed T and B lymphocytes, scattered macrophages and abundant polytypic plasma cells (kappa and lambda immunostains not shown). A moderate number of eosinophils are present. There is focal fibrosis showing a whorled (storiform) pattern. (C) The abundance of plasma cells is confirmed by CD138 immunostaining, x 200. (D) Immunostaining for IgG4. Forty per cent of the IgG+ plasma cells are IgG4+. There were >100 IgG4+ plasma cells per three high power fields. This biopsy specimen therefore meets criteria for IgG4 disease.