{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T01:15:21Z","timestamp":1760231721823,"version":"build-2065373602"},"reference-count":25,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2022,5,9]],"date-time":"2022-05-09T00:00:00Z","timestamp":1652054400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antibodies"],"abstract":"<jats:p>Early diagnosis and initiation of appropriate immunosuppressive treatment remain the cornerstone of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis at the cost of significant toxicity. In this report, we present a case of a 69-year-old female who presented with advanced renal insufficiency and evidence of pulmonary hemorrhage and was MPO\u2013ANCA-positive with a clinical phenotype of granulomatosis with polyangiitis. Organ involvement included rapidly progressive glomerulonephritis (GN), along with extrarenal manifestations (skin, upper and lower respiratory system involvement, and onset of saddle-nose deformity). Kidney biopsy established the diagnosis of pauci-immune crescentic, sclerotic GN. She received therapy with glucocorticoids and cyclophosphamide, mainly due to life-threatening extra-renal manifestations, such as pulmonary hemorrhage. She avoided vasculitis-related death but she developed severe therapy-related toxicity, resulting in the discontinuation of immunosuppressive therapy. Continuous re-evaluation of patients with ANCA-associated vasculitis in terms of response to immunosuppressive therapy and treatment-related toxicity is crucial for their management.<\/jats:p>","DOI":"10.3390\/antib11020033","type":"journal-article","created":{"date-parts":[[2022,5,9]],"date-time":"2022-05-09T21:23:27Z","timestamp":1652131407000},"page":"33","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["MPO\u2013ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report"],"prefix":"10.3390","volume":"11","author":[{"given":"Dimitra","family":"Petrou","sequence":"first","affiliation":[{"name":"Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Minas","family":"Karagiannis","sequence":"additional","affiliation":[{"name":"Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petros","family":"Nikolopoulos","sequence":"additional","affiliation":[{"name":"Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"George","family":"Liapis","sequence":"additional","affiliation":[{"name":"Department of Pathology, National and Kapodistrian University of Athens, Laiko Hospital, 11527 Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1643-8923","authenticated-orcid":false,"given":"Sophia","family":"Lionaki","sequence":"additional","affiliation":[{"name":"Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,9]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1046\/j.1523-1755.2003.00843.x","article-title":"Rapidly progressive crescentic glomerulonephritis","volume":"63","author":"Jennette","year":"2003","journal-title":"Kidney Int."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1053\/ajkd.1998.v32.pm9669443","article-title":"Crescentic glomerulonephritis with a paucity of glomerular immunoglobulin localization","volume":"32","author":"Harris","year":"1998","journal-title":"Am. 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