{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T07:48:12Z","timestamp":1782719292849,"version":"3.54.5"},"reference-count":58,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T00:00:00Z","timestamp":1782691200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>ANCA-associated vasculitis (AAV) with kidney involvement represents small-vessel vasculitis, characterized by rapidly progressive glomerulonephritis and a high risk of end-stage kidney disease (ESKD) and increased mortality. AAV typically presents with multisystem involvement, with renal manifestations occurring more frequently in microscopic polyangiitis (MPA) (90\u2013100%) and granulomatosis with polyangiitis (GPA) (50\u201380%). The classic clinical presentation includes acute kidney injury with hematuria and proteinuria, accompanied by ANCA positivity (MPO-ANCA or PR3-ANCA). Histologically, the predominant pattern is segmental necrotizing glomerulonephritis with crescent formation. Treatment consists of two phases: (a) induction of remission with a lower cumulative dose of glucocorticoids (according to the reduced-dose PEXIVAS regimen) in combination with rituximab or cyclophosphamide and (b) maintenance of remission with rituximab for 2\u20134 years. The C5a receptor inhibitor avacopan can be used as a steroid-sparing agent in patients with severe kidney involvement or at high risk of corticosteroid-related complications. Beyond the traditional markers of disease activity (hematuria, proteinuria, eGFR), novel biomarkers such as urinary soluble CD163, MCP-1, complement activation products (C5a, sC5b-9), and urinary Treg\/Th17 profiles have demonstrated prognostic value. Early diagnosis and prompt initiation of immunosuppressive therapy significantly improve both kidney and overall survival, while prevention of relapses and long-term complications plays a key role in improving the long-term prognosis of patients with AAV.<\/jats:p>","DOI":"10.3390\/medicina62071252","type":"journal-article","created":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T05:49:33Z","timestamp":1782712173000},"page":"1252","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Advances in the Diagnosis, Treatment and Prognosis of ANCA-Associated Glomerulonephritis"],"prefix":"10.3390","volume":"62","author":[{"given":"Aglaia","family":"Chalkia","sequence":"first","affiliation":[{"name":"Nephrology Department, General Hospital of Athens Hippokration, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dimitrios","family":"Petras","sequence":"additional","affiliation":[{"name":"Nephrology Department, General Hospital of Athens Hippokration, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"102495","DOI":"10.1016\/j.autrev.2020.102495","article-title":"Clinical associations of renal involvement in ANCA-associated vasculitis","volume":"19","author":"Kronbichler","year":"2020","journal-title":"Autoimmun. 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