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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MDPI AG
2022-06-01
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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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language | English |
last_indexed | 2024-03-09T03:32:42Z |
publishDate | 2022-06-01 |
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series | Diagnostics |
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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