{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T05:07:53Z","timestamp":1772168873169,"version":"3.50.1"},"update-to":[{"DOI":"10.12688\/f1000research.133102.1","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000}},{"DOI":"10.12688\/f1000research.133102.2","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000}},{"DOI":"10.12688\/f1000research.133102.3","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000}},{"DOI":"10.12688\/f1000research.133102.4","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000}}],"reference-count":16,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns4:p>\n                    <ns4:bold>Aim:<\/ns4:bold>\n                    We are reporting a case of an unusual presentation of granulomatosis with polyangiitis (GPA) with liver involvement.\n                    <ns4:bold>Case presentation:<\/ns4:bold>\n                    A 45-year-old male patient presented with erythematous plaques on the face and bilateral nasal obstruction. On physical examination, the patient had a ring-shaped squamous plaque on the face. The laboratory findings revealed an accelerated erythrocyte sedimentation rate at 100 mm\/h, an elevated C-reactive protein at 66 mg\/L, hyper gamma globulinemia 16 g\/L and an elevated alkaline phosphatase (twice the upper normal limit). The craniofacial and thoracoabdominal computed tomography (CT) -scans showed ethmoid and maxillary sinusitis, low facial bone density, multiple mediastinal and hilar lymphadenopathy, diffuse small pulmonary nodules, and hepatomegaly. A cutaneous lesion biopsy, the nasal mucosa, and the liver showed a chronic inflammatory granulomatosis process with necrosis. Serum anti-neutrophil cytoplasmic antibody (ANCA) against PR3 was positive. The clinical, biological, radiological, and histological findings substantiated the diagnosis of GPA. The patient received systemic steroids combined with cyclophosphamide pulses on days 1, 14 and 28 and then he was lost to follow-up. Two-years later, he presented with a cardiac failure and skin ulcer in the right lower limb. A nasal endoscopic exam showed nasal septum cartilage perforation with resorption of the middle and inferior nasal concha. Two weeks later, he developed a diffuse alveolar hemorrhage and was therefore transferred to the intensive care unit but died of respiratory failure 3 days later.\n                    <ns4:bold>Conclusion:<\/ns4:bold>\n                    Clinicians should be aware of GPA atypical clinical manifestations.\n                  <\/ns4:p>","DOI":"10.12688\/f1000research.133102.5","type":"journal-article","created":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T11:55:14Z","timestamp":1698062114000},"page":"430","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Case report: An unusual presentation of granulomatosis with polyangiitis"],"prefix":"10.12688","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0956-6348","authenticated-orcid":false,"given":"Ichrak","family":"Bannour","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1967-0824","authenticated-orcid":false,"given":"Maroi","family":"Ben Brahim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7797-4899","authenticated-orcid":false,"given":"Sondes","family":"Arfa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Soumaya ben","family":"Amor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7150-9515","authenticated-orcid":false,"given":"Asma","family":"Ben Mabrouk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olfa","family":"Berrich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonia","family":"Hammemi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2023,10,23]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1121-1125","DOI":"10.1016\/j.autrev.2014.08.017","article-title":"Granulomatosis with polyangiitis (Wegener): Clinical aspects and treatment.","volume":"13","author":"C Comarmond","year":"2014 Nov","journal-title":"Autoimmun. Rev."},{"key":"ref2","doi-asserted-by":"publisher","first-page":"8-13","DOI":"10.21053\/ceo.2016.9.1.8","article-title":"Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge.","volume":"9","author":"J Wojciechowska","year":"2016 Mars","journal-title":"Clin. Exp. Otorhinolaryngol."},{"key":"ref3","doi-asserted-by":"publisher","first-page":"151-159","DOI":"10.1177\/0394632015617063","article-title":"Clinic manifestations in granulomatosis with polyangiitis.","volume":"29","author":"A Greco","year":"2016 June","journal-title":"Int. J. Immunopathol. Pharmacol."},{"key":"ref4","doi-asserted-by":"publisher","first-page":"471-477","DOI":"10.1016\/j.ejrad.2004.04.016","article-title":"Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature.","volume":"53","author":"C Lohrmann","year":"2005 Mars","journal-title":"Eur. J. Radiol."},{"key":"ref5","doi-asserted-by":"publisher","first-page":"387-394","DOI":"10.1007\/s10067-015-2882-5","article-title":"Liver involvement in ANCA-associated vasculitis.","volume":"35","author":"P Willeke","year":"2016 Feb","journal-title":"Clin. Rheumatol."},{"key":"ref6","first-page":"1299-1306","article-title":"Outcome and prognostic factors during the course of primary small-vessel vasculitides.","volume":"33","author":"L Pavone","year":"2006 Jul 1","journal-title":"J. Rheumatol."},{"key":"ref7","first-page":"S88-S89","article-title":"Wegener\u2019s granulomatosis with granulomatous liver involvement.","volume":"28","author":"K Holl-Ulrich","year":"2010","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.20517\/2574-1209.2021.40","article-title":"Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis.","author":"M Abdel-Halim","year":"2022 cited 2022 Mar 14","journal-title":"Vessel Plus."},{"key":"ref9","doi-asserted-by":"publisher","first-page":"913667","DOI":"10.3389\/fimmu.2022.913667","article-title":"Long-Term Prognostic Factors in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A 15-Year Multicenter Retrospective Study.","volume":"13","author":"Q Liao","year":"2022 Jun 30","journal-title":"Front. Immunol."},{"key":"ref10","doi-asserted-by":"publisher","first-page":"340-349","DOI":"10.1097\/MD.0000000000000217","article-title":"Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature.","volume":"93","author":"C Comarmond","year":"2014 Nov","journal-title":"Medicine (Baltimore)."},{"key":"ref11","doi-asserted-by":"publisher","first-page":"947-956","DOI":"10.1093\/rheumatology\/kew490","article-title":"Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients.","volume":"56","author":"G Pugnet","year":"2017 Jun 1","journal-title":"Rheumatology."},{"key":"ref12","doi-asserted-by":"publisher","first-page":"1209-1212","DOI":"10.3899\/jrheum.141513","article-title":"Cardiac Involvement in Granulomatosis with Polyangiitis.","volume":"42","author":"L McGeoch","year":"2015 Jul","journal-title":"J. Rheumatol."},{"key":"ref13","doi-asserted-by":"publisher","first-page":"126-136","DOI":"10.1016\/j.case.2020.11.008","article-title":"Granulomatosis With Polyangiitis (Wegener\u2019s Granulomatosis) Complicated by Pericarditis: Our Experience of Two Cases and Comparative Review of Literature.","volume":"5","author":"T Ahmed","year":"2021 Apr","journal-title":"CASE."},{"key":"ref14","doi-asserted-by":"publisher","first-page":"386-390","DOI":"10.5935\/0103-507X.20170052","article-title":"Heart conduction system defects and sustained ventricular tachycardia complications in a patient with granulomatosis with polyangiitis. A case report and literature review.","volume":"29","author":"L Santos","year":"2017","journal-title":"Rev. Bras. Ter. Intensiva."},{"key":"ref15","doi-asserted-by":"publisher","first-page":"e6024","DOI":"10.1097\/MD.0000000000006024","article-title":"Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse.","volume":"96","author":"J Vanoli","year":"2017","journal-title":"Medicine (Baltimore)."},{"key":"ref16","article-title":"Granulomatosis With Polyangiitis. [Updated 2022 Dec 5].","author":"P Garlapati","year":"2023 Jan"}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/12-430\/v5\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/12-430\/v5\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/12-430\/v5\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,25]],"date-time":"2023-10-25T06:11:07Z","timestamp":1698214267000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/12-430\/v5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,23]]},"references-count":16,"URL":"https:\/\/doi.org\/10.12688\/f1000research.133102.5","relation":{"has-review":[{"id-type":"doi","id":"10.5256\/f1000research.157826.r217613","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.157826.r217613","asserted-by":"object"}]},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,23]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/12-430\/v5#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.152226.r188590, Gloria Vasquez, Rheumatology Section, Facultad de Medicina, Universidad de Antioquia, Medell\u00edn, Antioquia, 20 Jul 2023, version 3, 1 approved","URL":"https:\/\/f1000research.com\/articles\/12-430\/v3#referee-response-188590","order":0,"name":"referee-response-188590","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"<b>Ichrak Bannour<\/b>; \n<i>Posted: 20 Jul 2023<\/i>; I do want to thank the reviewer Professor Gloria Vasquez for her corrections and suggestions of improvement. I corrected the typewriting mistake and omitted the expression &quot;pulmonary fibrosis&quot;","URL":"https:\/\/f1000research.com\/articles\/12-430\/v5#referee-comment-9938","order":1,"name":"referee-comment-9938","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.157826.r217613, Meriem Kallel, Immuno-Rheumatology Research Laboratory, Rheumatology Department, La Rabta Hospital, University of Tunis-El Manar, Tunis, 25 Oct 2023, version 5, indexed","URL":"https:\/\/f1000research.com\/articles\/12-430\/v5#referee-response-217613","order":2,"name":"referee-response-217613","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The author(s) declared that no grants were involved in supporting this work","order":3,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.","order":0,"name":"copyright-info","label":"Copyright"}]}}