RT Journal Article SR Electronic T1 IgG4-related disease presenting as posterior scleritis and vitritis, progressing to multifocal orbital involvement JF BMJ Case Reports FD BMJ Publishing Group Ltd SP bcr-2017-219568 DO 10.1136/bcr-2017-219568 VO 2017 A1 Gavin L Reynolds A1 Jonathan H Norris A1 Sher Aslam A1 Srilakshmi Sharma YR 2017 UL http://casereports.bmj.com/content/2017/bcr-2017-219568.abstract AB 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.