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Ocular Involvement of Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener’s disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented...

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Autores principales: Byszewska, Anna, Skrzypiec, Izabela, Rymarz, Aleksandra, Niemczyk, Stanisław, Rękas, Marek
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10342598/
https://www.ncbi.nlm.nih.gov/pubmed/37445483
http://dx.doi.org/10.3390/jcm12134448
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author Byszewska, Anna
Skrzypiec, Izabela
Rymarz, Aleksandra
Niemczyk, Stanisław
Rękas, Marek
author_facet Byszewska, Anna
Skrzypiec, Izabela
Rymarz, Aleksandra
Niemczyk, Stanisław
Rękas, Marek
author_sort Byszewska, Anna
collection PubMed
description Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener’s disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented with comprehensive photographic documentation. We describe the way to proper diagnosis, which may be long, the possible treatment, and the final outcomes. Our patients had the following ocular manifestations of GPA: retinal vasculitis, anterior necrotizing scleritis, medial orbital wall and orbital floor erosion with middle face deformation, compressive optic neuropathy due to retrobulbar inflammatory mass, and the abscess of the eyelids, inflammatory intraorbital mass causing exophthalmos and diplopia. This manuscript includes the description of severe forms of GPA, the initial signs and symptoms, relapses, and difficulties in achieving remission. The extraocular involvement is described with diagnostic modalities and laboratory findings. One of the reported cases was diagnosed by an ophthalmologist on the basis of ocular symptoms in the early stages of the disease. Our outcomes are compared with those discussed in the literature.
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spelling pubmed-103425982023-07-14 Ocular Involvement of Granulomatosis with Polyangiitis Byszewska, Anna Skrzypiec, Izabela Rymarz, Aleksandra Niemczyk, Stanisław Rękas, Marek J Clin Med Article Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener’s disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented with comprehensive photographic documentation. We describe the way to proper diagnosis, which may be long, the possible treatment, and the final outcomes. Our patients had the following ocular manifestations of GPA: retinal vasculitis, anterior necrotizing scleritis, medial orbital wall and orbital floor erosion with middle face deformation, compressive optic neuropathy due to retrobulbar inflammatory mass, and the abscess of the eyelids, inflammatory intraorbital mass causing exophthalmos and diplopia. This manuscript includes the description of severe forms of GPA, the initial signs and symptoms, relapses, and difficulties in achieving remission. The extraocular involvement is described with diagnostic modalities and laboratory findings. One of the reported cases was diagnosed by an ophthalmologist on the basis of ocular symptoms in the early stages of the disease. Our outcomes are compared with those discussed in the literature. MDPI 2023-07-02 /pmc/articles/PMC10342598/ /pubmed/37445483 http://dx.doi.org/10.3390/jcm12134448 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Byszewska, Anna
Skrzypiec, Izabela
Rymarz, Aleksandra
Niemczyk, Stanisław
Rękas, Marek
Ocular Involvement of Granulomatosis with Polyangiitis
title Ocular Involvement of Granulomatosis with Polyangiitis
title_full Ocular Involvement of Granulomatosis with Polyangiitis
title_fullStr Ocular Involvement of Granulomatosis with Polyangiitis
title_full_unstemmed Ocular Involvement of Granulomatosis with Polyangiitis
title_short Ocular Involvement of Granulomatosis with Polyangiitis
title_sort ocular involvement of granulomatosis with polyangiitis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10342598/
https://www.ncbi.nlm.nih.gov/pubmed/37445483
http://dx.doi.org/10.3390/jcm12134448
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