{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T05:16:40Z","timestamp":1792819000700,"version":"4.5.0"},"reference-count":21,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2024,9,23]],"date-time":"2024-09-23T00:00:00Z","timestamp":1727049600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Japan Society for the Promotion of Science (JSPS) KAKENHI","award":["19\u2009K18837 (SN)"],"award-info":[{"award-number":["19\u2009K18837 (SN)"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and objectives: The initial symptom that triggers granulomatosis with polyangiitis (GPA) diagnosis is rarely ocular. We describe a case with a single ocular lesion identified as probable GPA due to proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-positivity according to the diagnostic criteria of the Ministry of Health in Japan; the lesion repeatedly worsened. Materials and methods: A 25-year-old female visited the Department of Ophthalmology, Asahi General Hospital, with upper eyelid swelling and conjunctival and episcleral hyperemia of the left eye. Both hordeolum and eyelid cellulitis were suspected, as the condition was resistant to treatment with antibiotic eye drops. Episcleritis was suspected due to localized hyperemia in the upper part of the eye. Upon treatment with antibacterial agents and steroid eye drops, the swelling and the hyperemia repeatedly worsened every week. Results: Blood samples were positive for PR3-ANCA, and GPA with an isolated ocular lesion was considered. After oral steroid treatment, the patient had no recurrence for 4 years. There was no systemic involvement in the upper respiratory tract, lungs, or kidneys. Conclusions: Diagnosing GPA with ocular symptoms as initial manifestations is challenging. GPA should be considered in treatment-resistant eyelid, orbital, and episcleral lesions, even at a young age.<\/jats:p>","DOI":"10.3390\/medicina60091555","type":"journal-article","created":{"date-parts":[[2024,9,23]],"date-time":"2024-09-23T03:36:38Z","timestamp":1727062598000},"page":"1555","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Granulomatosis with Polyangiitis Discovered Because of Repeated Upper Eyelid Swelling"],"prefix":"10.3390","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9938-4881","authenticated-orcid":false,"given":"Suguru","family":"Nakagawa","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, Asahi General Hospital, Chiba 289-2511, Japan"},{"name":"Department of Ophthalmology, Saitama Medical Center, Jichi Medical University, Saitama 330-0834, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiyohito","family":"Totsuka","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Asahi General Hospital, Chiba 289-2511, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichiro","family":"Kagami","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Asahi General Hospital, Chiba 289-2511, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yohei","family":"Nomoto","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Asahi General Hospital, Chiba 289-2511, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1056\/NEJM195304302481805","article-title":"Periarteritis nodosa and other forms of necrotizing angiitis","volume":"248","author":"Zeek","year":"1953","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1016\/j.rdc.2010.04.002","article-title":"Epidemiology of ANCA-associated vasculitis","volume":"36","author":"Ntatsaki","year":"2010","journal-title":"Rheum. Dis. Clin."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"61","DOI":"10.5582\/irdr.2016.01014","article-title":"Granulomatosis with polyangiitis (Wegener\u2019s disease): An updated review of ocular disease manifestations","volume":"5","author":"Kubaisi","year":"2016","journal-title":"Intractable Rare Dis. Res."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1097\/00002281-200101000-00002","article-title":"Management of the ear, nose, and throat manifestations of Wegener granulomatosis: An otorhinolaryngologist\u2019s perspective","volume":"13","author":"Rasmussen","year":"2001","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3","DOI":"10.22336\/rjo.2020.2","article-title":"Eye involvement in ANCA positive vasculitis","volume":"64","author":"Macarie","year":"2020","journal-title":"Rom. J. Ophthalmol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1322","DOI":"10.1002\/art.1780351113","article-title":"The treatment of Wegener\u2019s granulomatosis with glucocorticoids and methotrexate","volume":"35","author":"Hoffman","year":"1992","journal-title":"Arthritis Rheum."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"R101","DOI":"10.1186\/ar4550","article-title":"Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study","volume":"16","author":"Sada","year":"2014","journal-title":"Arthritis Res. Ther."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"657","DOI":"10.3109\/14397595.2014.982270","article-title":"Issues associated with the Ministry of Health, Labour and Welfare diagnostic criteria for antineutrophil cytoplasmic antibody-associated vasculitides: Reclassification of patients in the prospective cohort study of Remission Induction Therapy in Japanese patients with ANCA-associated vasculitides according to the MHLW criteria","volume":"25","author":"Sada","year":"2015","journal-title":"Mod. Rheumatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1093\/mr\/road052","article-title":"Evaluation of Ministry of Health, Labour and Welfare diagnostic criteria for antineutrophil cytoplasmic antibody\u2013associated vasculitis compared to ACR\/EULAR 2022 classification criteria","volume":"34","author":"Sada","year":"2024","journal-title":"Mod. Rheumatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1002\/art.41986","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis","volume":"74","author":"Robson","year":"2022","journal-title":"Arthritis Rheumatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1515","DOI":"10.1007\/s00417-022-05918-w","article-title":"Updates of ocular involvement in granulomatosis with polyangiitis","volume":"261","author":"Mei","year":"2023","journal-title":"Graefe\u2019s Arch. Clin. Exp. Ophthalmol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"iii42","DOI":"10.1093\/rheumatology\/keaa089","article-title":"An update on the epidemiology of ANCA-associated vasculitis","volume":"59","author":"Mohammad","year":"2020","journal-title":"Rheumatology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1007\/s40123-019-0176-8","article-title":"Ocular manifestations of granulomatosis with polyangiitis: A review of the literature","volume":"8","author":"Sfiniadaki","year":"2019","journal-title":"Ophthalmol. Ther."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Byszewska, A., Skrzypiec, I., Rymarz, A., Niemczyk, S., and R\u0119kas, M. (2023). Ocular involvement of granulomatosis with polyangiitis. J. Clin. Med., 12.","DOI":"10.3390\/jcm12134448"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1751","DOI":"10.1007\/s00417-018-4014-9","article-title":"Clinical features of different orbital manifestations of granulomatosis with polyangiitis","volume":"256","author":"Ismailova","year":"2018","journal-title":"Graefe\u2019s Arch. Clin. Exp. Ophthalmol."},{"key":"ref_16","first-page":"214","article-title":"Ocular and orbital manifestations of granulomatosis with polyangiitis: A systematic review of published cases","volume":"67","author":"Moin","year":"2023","journal-title":"Rom. J. Ophthalmol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1017\/S0022215106005032","article-title":"Patterns of presentation and diagnosis of patients with Wegener\u2019s granulomatosis: ENT aspects","volume":"121","author":"Srouji","year":"2007","journal-title":"J. Laryngol. Otol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1038\/s41572-020-0204-y","article-title":"ANCA-associated vasculitis","volume":"6","author":"Kitching","year":"2020","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_19","first-page":"e37","article-title":"Granulomatosis with polyangiitis (Wegener\u2019s granulomatosis) in children: Report of three cases with cutaneous manifestations and literature review","volume":"30","author":"Nikolic","year":"2013","journal-title":"Pediatr. Dermatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1001\/archopht.1971.01000010519006","article-title":"Granulomatous sclerouveitis: Regression of ocular lesions with cyclophosphamide and prednisone","volume":"86","author":"Brubaker","year":"1971","journal-title":"Arch. Ophthalmol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/S0161-6420(83)34574-7","article-title":"Ocular complications of Wegener\u2019s granulomatosis","volume":"90","author":"Bullen","year":"1983","journal-title":"Ophthalmology"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/9\/1555\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:02:31Z","timestamp":1760112151000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/9\/1555"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,23]]},"references-count":21,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2024,9]]}},"alternative-id":["medicina60091555"],"URL":"https:\/\/doi.org\/10.3390\/medicina60091555","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,23]]}}}