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...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2015
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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. |
format | Online Article Text |
id | pubmed-4446498 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
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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