{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T16:48:46Z","timestamp":1784047726539,"version":"3.55.0"},"reference-count":36,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2021,8,19]],"date-time":"2021-08-19T00:00:00Z","timestamp":1629331200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Perinuclear anti-neutrophilic cytoplasmic antibodies (P-ANCA) recognize heterogeneous antigens, including myeloperoxidase (MPO), lactoferrin, elastase, cathepsin-G and bactericidal\/permeability-increasing protein. Although P-ANCA have diagnostic utility in vasculitides, they may also be found in patients with various other systemic autoimmune rheumatic diseases (SARDs). Nevertheless, the clinical significance and the targets recognized by P-ANCA in such patients remain unclear. For this purpose, herein we investigated the occurrence of ANCA-related antigenic specificities in 82 P-ANCA-positive sera by multiplex ELISA, as well as their association with other autoantibodies. The P-ANCA-positive sera corresponded to patients with vasculitides (n = 24), systemic lupus erythematosus (n = 28), antiphospholipid syndrome (n = 5), Sj\u00f6gren\u2019s syndrome (n = 7), rheumatoid arthritis (n = 3), systemic scleroderma (n = 1), sarcoidosis (n = 1) and Hashimoto\u2032s thyroiditis (n = 13). In most P-ANCA-positive patients studied (51\/82, 62.3%), these autoantibodies occurred in high titers (&gt;1:160). The analysis of P-ANCA-positive sera revealed reactivity to MPO in only 50% of patients with vasculitides, whereas it was infrequent in the other disease groups studied. Reactivity to other P-ANCA-related autoantigens was also rarely detected. Our findings support that high P-ANCA titers occur in SARD. The P-ANCA-positive staining pattern is associated with MPO specificity in vasculitides, while in other autoimmune diseases, it mostly involves unknown autoantigens.<\/jats:p>","DOI":"10.3390\/cells10082128","type":"journal-article","created":{"date-parts":[[2021,8,19]],"date-time":"2021-08-19T04:13:54Z","timestamp":1629346434000},"page":"2128","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Occurrence and Antigenic Specificity of Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (P-ANCA) in Systemic Autoimmune Diseases"],"prefix":"10.3390","volume":"10","author":[{"given":"Ourania D.","family":"Argyropoulou","sequence":"first","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4343-0396","authenticated-orcid":false,"given":"Andreas V.","family":"Goules","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgios","family":"Boutzios","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Tsirogianni","sequence":"additional","affiliation":[{"name":"Department of Immunology and Histocompatibility, Evangelismos General Hospital, 10676 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charalampos","family":"Sfontouris","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Evangelismos General Hospital, 10676 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6415-0946","authenticated-orcid":false,"given":"Menelaos N.","family":"Manoussakis","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panayiotis G.","family":"Vlachoyiannopoulos","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Athanasios G.","family":"Tzioufas","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Efstathia K.","family":"Kapsogeorgou","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"},{"name":"Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.berh.2004.10.003","article-title":"What do antineutrophil cytoplasmic antibodies (ANCA) tell us?","volume":"19","author":"Savige","year":"2005","journal-title":"Best Pract. 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