Reynolds Gavin L, Norris Jonathan H, Aslam Sher, Sharma Srilakshmi
Oxford Eye Hospital, John Radcliffe Hospital, Oxford, UK.
BMJ Case Rep. 2017 Apr 23;2017:bcr-2017-219568. doi: 10.1136/bcr-2017-219568.
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.
IgG4相关性疾病（IgG4-RD）是一种罕见的慢性炎症性疾病，可能累及几乎每个器官系统。最初被确定为自身免疫性胰腺炎的病因，现已在包括眼和眼眶在内的多种炎症表现中发现其特征性的组织学和临床特征。在此，我们描述一例最初表现为巩膜炎和葡萄膜炎，进而发展为多灶性双侧眼眶受累的IgG4-RD病例。眼眶肿物的组织活检具有IgG4-RD组织学的高度特征性，并且观察到对皮质类固醇有快速的临床反应。该病例突出了IgG4-RD作为一种罕见的眼内炎症病因，可能进展累及眼眶。
