{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:50:37Z","timestamp":1787028637418,"version":"build-2736575974"},"reference-count":23,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2023,7,2]],"date-time":"2023-07-02T00:00:00Z","timestamp":1688256000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Military Institute of Medicine- National Research Institute","award":["626"],"award-info":[{"award-number":["626"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener\u2019s 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.<\/jats:p>","DOI":"10.3390\/jcm12134448","type":"journal-article","created":{"date-parts":[[2023,7,3]],"date-time":"2023-07-03T00:49:27Z","timestamp":1688345367000},"page":"4448","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Ocular Involvement of Granulomatosis with Polyangiitis"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6143-9004","authenticated-orcid":false,"given":"Anna","family":"Byszewska","sequence":"first","affiliation":[{"name":"Ophthalmology Department, Military Institute of Medicine\u2013National Research Institute, Szaser\u00f3w 128, 04-141 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3210-2023","authenticated-orcid":false,"given":"Izabela","family":"Skrzypiec","sequence":"additional","affiliation":[{"name":"Ophthalmology Department, Military Institute of Medicine\u2013National Research Institute, Szaser\u00f3w 128, 04-141 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1844-9872","authenticated-orcid":false,"given":"Aleksandra","family":"Rymarz","sequence":"additional","affiliation":[{"name":"Nephrology Department, Military Institute of Medicine\u2013National Research Institute, Szaser\u00f3w 128, 04-141 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanis\u0142aw","family":"Niemczyk","sequence":"additional","affiliation":[{"name":"Nephrology Department, Military Institute of Medicine\u2013National Research Institute, Szaser\u00f3w 128, 04-141 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0429-6649","authenticated-orcid":false,"given":"Marek","family":"R\u0119kas","sequence":"additional","affiliation":[{"name":"Ophthalmology Department, Military Institute of Medicine\u2013National Research Institute, Szaser\u00f3w 128, 04-141 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2945","DOI":"10.1002\/art.39812","article-title":"Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients with Granulomatosis with Polyangiitis (Wegener\u2019s): Distinct Patient Subsets","volume":"68","author":"Miloslavsky","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"643.e9","DOI":"10.1016\/j.amjmed.2006.08.016","article-title":"ANCA Are Detectable in Nearly All Patients with Active Severe Wegener\u2019s Granulomatosis","volume":"120","author":"Finkielman","year":"2007","journal-title":"Am. 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