Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex

Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease consisting of necrotizing granulomatosis of the respiratory tract, necrotizing vasculitis, and necrotizing glomerulonephritis. It is under the category of ANCA-associated vasculitis, which involves small vessels. The nose, sinus...

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Main Authors: Shang-Yen Wu, Ling-Shuo Chang, Yuan-Chieh Lee, Yu-Jen Pan, Yu-Fu Chou, Fang-Ling Chang, Yu-Hsuan Lu, Nancy Chen
Format: Article
Language:English
Published: MDPI AG 2022-06-01
Series:Diagnostics
Subjects:
Online Access:https://www.mdpi.com/2075-4418/12/7/1540
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author Shang-Yen Wu
Ling-Shuo Chang
Yuan-Chieh Lee
Yu-Jen Pan
Yu-Fu Chou
Fang-Ling Chang
Yu-Hsuan Lu
Nancy Chen
author_facet Shang-Yen Wu
Ling-Shuo Chang
Yuan-Chieh Lee
Yu-Jen Pan
Yu-Fu Chou
Fang-Ling Chang
Yu-Hsuan Lu
Nancy Chen
author_sort Shang-Yen Wu
collection DOAJ
description Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease consisting of necrotizing granulomatosis of the respiratory tract, necrotizing vasculitis, and necrotizing glomerulonephritis. It is under the category of ANCA-associated vasculitis, which involves small vessels. The nose, sinus, and ear were the most affected sites besides lung and kidney in localized form. They might precede other disease manifestations before progressing to the systemic form. Our patient presented with an intractable headache, followed by acute vision loss. His symptoms deteriorated regardless of antibiotic treatment for paranasal sinusitis. The sequential CT/MRI images showed the inflammatory raid of the orbital apex and cavernous sinus within days. The sinus biopsy and elevated PR3-anti-neutrophil cytoplasmic antibody led us to the diagnosis of GPA. Fortunately, the patient’s vision improved gradually after steroid and immunosuppressant treatment.
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spelling doaj.art-ae4b555269ba4aef811ebf86e53d39502023-12-03T14:53:30ZengMDPI AGDiagnostics2075-44182022-06-01127154010.3390/diagnostics12071540Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital ApexShang-Yen Wu0Ling-Shuo Chang1Yuan-Chieh Lee2Yu-Jen Pan3Yu-Fu Chou4Fang-Ling Chang5Yu-Hsuan Lu6Nancy Chen7Department of Ophthalmology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDepartment of Ophthalmology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDepartment of Ophthalmology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDivision of Allergy, Immunology & Rheumatology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDepartment of Otolaryngology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDepartment of Ophthalmology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDepartment of Ophthalmology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanDepartment of Ophthalmology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 970, TaiwanGranulomatosis with polyangiitis (GPA) is a systemic autoimmune disease consisting of necrotizing granulomatosis of the respiratory tract, necrotizing vasculitis, and necrotizing glomerulonephritis. It is under the category of ANCA-associated vasculitis, which involves small vessels. The nose, sinus, and ear were the most affected sites besides lung and kidney in localized form. They might precede other disease manifestations before progressing to the systemic form. Our patient presented with an intractable headache, followed by acute vision loss. His symptoms deteriorated regardless of antibiotic treatment for paranasal sinusitis. The sequential CT/MRI images showed the inflammatory raid of the orbital apex and cavernous sinus within days. The sinus biopsy and elevated PR3-anti-neutrophil cytoplasmic antibody led us to the diagnosis of GPA. Fortunately, the patient’s vision improved gradually after steroid and immunosuppressant treatment.https://www.mdpi.com/2075-4418/12/7/1540granulomatosis with polyangiitisvision lossparanasal sinusitisPR3-anti-neutrophil cytoplasmic antibody
spellingShingle Shang-Yen Wu
Ling-Shuo Chang
Yuan-Chieh Lee
Yu-Jen Pan
Yu-Fu Chou
Fang-Ling Chang
Yu-Hsuan Lu
Nancy Chen
Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex
Diagnostics
granulomatosis with polyangiitis
vision loss
paranasal sinusitis
PR3-anti-neutrophil cytoplasmic antibody
title Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex
title_full Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex
title_fullStr Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex
title_full_unstemmed Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex
title_short Acute Vision Loss as the Initial Manifestation of Granulomatosis with Polyangiitis Involving the Orbital Apex
title_sort acute vision loss as the initial manifestation of granulomatosis with polyangiitis involving the orbital apex
topic granulomatosis with polyangiitis
vision loss
paranasal sinusitis
PR3-anti-neutrophil cytoplasmic antibody
url https://www.mdpi.com/2075-4418/12/7/1540
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