IgG4-Related Orbital Inflammation

摘要: IgG4-related inflammation is a recently recognized fibro-inflammatory condition, characterized by tumefactive lesions containing dense lymphoplasmacytic infiltrates rich in IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis that are associated with elevated serum IgG4. Orbital adnexal tissues the fifth most common extrapancreatic site. It mostly seen middle-aged patients equal involvement of men women. can affect any tissue orbit, but lacrimal gland (88 %) commonly involved, followed trigeminal nerve branch enlargement (39 %), extraocular (25 diffuse infiltration orbital fat (23 %). Patients usually present mass or mass-related symptoms mild no signs inflammation. The diagnostic criteria include diffuse/localized masses single multiple organs, IgG4 concentrations (≥135 mg/dl), histopathological examination showing marked lymphocytes cell cells comprising more than 40 % total population at least 10 per high-power microscopic field. On magnetic resonance imaging, hyperintense on T1-weighted hypointense T2-weighted images. They enhance gadolinium have destructive bony lesion. thought to be particular Th-2-mediated inflammatory response may initiated autoantigens become during process. There delicate balance between Th-2 Treg evolving responses, behaves as surrogate marker type pro-fibrotic process which it serves mitigate response. shows good initial corticosteroids, disease evolves into sclerotic phase, becomes resistant. In these cases, immunosuppressants rituximab been used some success.