UNASSIGNED: Although chronic inflammatory disorders of the ocular adnexa are relatively common, their pathogenesis is in many cases poorly understood. Recent investigation suggests that many cases of @entity32 are a manifestation of IgG4-related disease; however, most @entity1 reported have been Asian, and it is not clear whether the results of studies from the Far East can be reliably extrapolated to draw conclusions about Western @entity1 . We evaluated 38 cases previously diagnosed as orbital inflammatory @entity6437 or @entity4578 to determine whether our cases fulfill the criteria for IgG4-RD (IgG4-related @entity4578 when involving the lacrimal gland, and IgG4-related @entity32 when involving orbital soft tissue). Fifteen patients had IgG4-related @entity32 . These @entity1 included 9 @entity1 and 6 @entity1 , aged 24 to 77 years (median, 64 y). Lesions involved orbital soft tissue (8 cases), lacrimal gland (6 cases), and canthus (1 case). In 1 case, focal in situ follicular @entity5 was seen in a background of IgG4-RD. In another case, a clonal @entity7513 gene rearrangement was detected. Four patients with IgG4-RD had evidence of IgG4-RD in other anatomic sites. Five @entity1 , 1 @entity1 and 4 @entity1 , aged 26 to 74 years (median 50 y) had orbital lesions (2 involving lacrimal gland, 3 involving soft tissue) suspicious for, but not diagnostic of, IgG4-RD. Of 16 patients with IgG4-RD or probable IgG4-RD with information available regarding the course of their disease, 11 @entity1 experienced recurrent or persistent @entity2694 . However, no @entity1 developed @entity706 , and no @entity1 died of complications of IgG4-RD. Eighteen @entity1 had lesions not representing IgG4-RD. They included 6 male and 12 female individuals aged 6 to 77 years (median, 47 y). These @entity1 had a variety of diseases, including @entity2883 with @entity2883 (3 cases), @entity9312 (1 case), nonspecific @entity32 and @entity82 involving lacrimal gland or soft tissue (12 cases), and others. Clinical and pathologic findings among our patients with IgG4-RD involving the orbit are similar to those previously described in Asian @entity1 . Careful evaluation of histologic and immunophenotypic features and clinical correlation are required to distinguish orbital IgG4-RD from other sclerosing inflammatory lesions in the orbit.

IgG4-related XXXX and Its Mimics in a Western Population.

@entity1 :: ('9606', 'Species') :: ['women', 'patients', 'patient', 'man', 'men'] @entity32 :: ('MESH:D007249', 'Disease') :: ['chronic inflammation', 'sclerosing orbital inflammation', 'dacryoadenitis or orbital inflammation'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrosis'] @entity9312 :: ('MESH:D015618', 'Disease') :: ['Rosai-Dorfman disease'] @entity706 :: ('MESH:D008223', 'Disease') :: ['lymphoma'] @entity5 :: ('MESH:D009369', 'Disease') :: ['neoplasia'] @entity6437 :: ('MESH:D006104', 'Disease') :: ['pseudotumor'] @entity2694 :: ('MESH:D009916', 'Disease') :: ['orbital disease'] @entity2883 :: ('MESH:D014890', 'Disease') :: ['polyangiitis', 'granulomatosis'] @entity7513 :: ('3492', 'Gene') :: ['IGH'] @entity4578 :: ('MESH:D003607', 'Disease') :: ['chronic dacryoadenitis', 'dacryoadenitis']
@entity2694 :: (MESH:D009916,Disease) :: ['Orbital Disease']
