IgG4-associated orbital and ocular inflammation

BACKGROUND: IgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopath...

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Autores principales: Lee, Cecilia S, Harocopos, George J, Kraus, Courtney L, Lee, Aaron Y, Van Stavern, Gregory P, Couch, Steven M, Rao, P Kumar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4446498/
https://www.ncbi.nlm.nih.gov/pubmed/26034515
http://dx.doi.org/10.1186/s12348-015-0047-y
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author Lee, Cecilia S
Harocopos, George J
Kraus, Courtney L
Lee, Aaron Y
Van Stavern, Gregory P
Couch, Steven M
Rao, P Kumar
author_facet Lee, Cecilia S
Harocopos, George J
Kraus, Courtney L
Lee, Aaron Y
Van Stavern, Gregory P
Couch, Steven M
Rao, P Kumar
author_sort Lee, Cecilia S
collection PubMed
description BACKGROUND: IgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopathologic evaluation. FINDINGS: We report three cases with differing manifestations of IgG4-associated ocular and orbital inflammation: a case of recurrent, treatment-refractory sclero-uveitis that was diagnosed as granulomatosis with polyangiitis with an IgG4-related component, a case of pachymeningitis with optic neuritis that resulted in permanent visual loss, and a case of orbital inflammatory pseudotumor. All three would have been incompletely diagnosed without thorough histopathologic evaluation (including immunohistochemistry). CONCLUSIONS: IgG4-associated disease is an idiopathic, multi-organ inflammatory state that can manifest as chronic, relapsing, sclerosing inflammation in virtually any organ system. There is a wide range of presentations in ocular and orbital inflammation. Ophthalmologists should keep IgG4-associated inflammation in mind when examining chronic, sclerofibrosing inflammation with multi-system involvement. The histology of biopsy specimens is crucial in making the correct diagnosis. Timely assessment may lead to fewer diagnostic tests and more targeted therapy.
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spelling pubmed-44464982015-06-01 IgG4-associated orbital and ocular inflammation Lee, Cecilia S Harocopos, George J Kraus, Courtney L Lee, Aaron Y Van Stavern, Gregory P Couch, Steven M Rao, P Kumar J Ophthalmic Inflamm Infect Brief Report BACKGROUND: IgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopathologic evaluation. FINDINGS: We report three cases with differing manifestations of IgG4-associated ocular and orbital inflammation: a case of recurrent, treatment-refractory sclero-uveitis that was diagnosed as granulomatosis with polyangiitis with an IgG4-related component, a case of pachymeningitis with optic neuritis that resulted in permanent visual loss, and a case of orbital inflammatory pseudotumor. All three would have been incompletely diagnosed without thorough histopathologic evaluation (including immunohistochemistry). CONCLUSIONS: IgG4-associated disease is an idiopathic, multi-organ inflammatory state that can manifest as chronic, relapsing, sclerosing inflammation in virtually any organ system. There is a wide range of presentations in ocular and orbital inflammation. Ophthalmologists should keep IgG4-associated inflammation in mind when examining chronic, sclerofibrosing inflammation with multi-system involvement. The histology of biopsy specimens is crucial in making the correct diagnosis. Timely assessment may lead to fewer diagnostic tests and more targeted therapy. Springer Berlin Heidelberg 2015-05-29 /pmc/articles/PMC4446498/ /pubmed/26034515 http://dx.doi.org/10.1186/s12348-015-0047-y Text en © Lee et al.; licensee Springer. 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited.
spellingShingle Brief Report
Lee, Cecilia S
Harocopos, George J
Kraus, Courtney L
Lee, Aaron Y
Van Stavern, Gregory P
Couch, Steven M
Rao, P Kumar
IgG4-associated orbital and ocular inflammation
title IgG4-associated orbital and ocular inflammation
title_full IgG4-associated orbital and ocular inflammation
title_fullStr IgG4-associated orbital and ocular inflammation
title_full_unstemmed IgG4-associated orbital and ocular inflammation
title_short IgG4-associated orbital and ocular inflammation
title_sort igg4-associated orbital and ocular inflammation
topic Brief Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4446498/
https://www.ncbi.nlm.nih.gov/pubmed/26034515
http://dx.doi.org/10.1186/s12348-015-0047-y
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