{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T07:55:47Z","timestamp":1791618947507,"version":"4.3.4"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2020,9,6]],"date-time":"2020-09-06T00:00:00Z","timestamp":1599350400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Although rapidly progressive glomerulonephritis is the main renal phenotype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), slow renal disease progression is sometimes observed. These forms have been rarely discussed; we analysed their prevalence, clinico-pathological characteristics and outcome.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We screened patients with microscopic \u00a0polyangiitis (MPA) and granulomatosis with polyangiitis followed at seven referral centres and selected those with estimated glomerular filtration rate (eGFR) reduction &amp;lt;50% over a 6-month period preceding diagnosis. Data regarding patient features and response to treatment were retrieved.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Of 856 patients, 41 (5%) had slowly progressive renal AAV. All had MPA and all but one was P-ANCA\/myeloperoxidase (MPO) ANCA-positive. At diagnosis, the median age was 70\u2009years [interquartile range (IQR) 64\u201378] and extra-renal manifestations were absent or subclinical (interstitial lung lesions in 10, 24%). The median (IQR) eGFR was 23\u2009mL\/min\/1.73\u2009m2 (15\u201335); six patients (15%) had started renal replacement therapy (RRT). All had proteinuria (median 1180\u2009mg\/24\u2009h, IQR 670\u20132600) and micro-haematuria. Main histologic findings were extracapillary proliferation at chronic stages and glomerulosclerosis; following Berden\u2019s classification, 6\/28 biopsies (21%) were \u2018focal\u2019, 1\/28 (4%) \u2018crescentic\u2019, 9\/28 (32%) \u2018mixed\u2019 and 12\/28 (43%) \u2018sclerotic\u2019. At last follow-up (median 32\u2009months, IQR 12\u201352), 20\/34 patients (59%) treated with immunosuppression had eGFR improvement &amp;gt;25% as compared with diagnosis, while 4\/34 (12%) had started RRT.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>AAV may present with slow renal disease progression; this subset is hallmarked by advanced age at diagnosis, positive MPO-ANCA, subclinical interstitial lung lesions and chronic damage at kidney biopsy. Partial renal recovery may occur following immunosuppression.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ckj\/sfaa139","type":"journal-article","created":{"date-parts":[[2020,6,22]],"date-time":"2020-06-22T11:28:25Z","timestamp":1592825305000},"page":"332-340","source":"Crossref","is-referenced-by-count":33,"title":["Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome"],"prefix":"10.1093","volume":"14","author":[{"given":"Giorgio","family":"Trivioli","sequence":"first","affiliation":[{"name":"Department of Biomedical Experimental and Clinical Sciences \u201cMario Serio\u201d, University of Firenze, and Nephrology Unit, Meyer Children\u2019s Hospital, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seerapani","family":"Gopaluni","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Cambridge, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria L.","family":"Urban","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Sciences, University of Firenze, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Gianfreda","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Fondazione Panico Hospital, Tricase, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias A.","family":"Cassia","sequence":"additional","affiliation":[{"name":"Department of Health Science, University of Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo G.","family":"Vercelloni","sequence":"additional","affiliation":[{"name":"Nephrology Unit, University Milano Bicocca, Monza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Calatroni","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Humanitas Hospital, Rozzano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Bettiol","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Psychology, Pharmacology and Child Health, University of Firenze, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasquale","family":"Esposito","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Medical Specialties, University of Genova, Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corrado","family":"Murtas","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Nephrology Unit, ASSL Oristano, ATS Sardegna, Oristano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Alberici","sequence":"additional","affiliation":[{"name":"Nephrology Unit, ASST Spedali Civili, Brescia, Italy"},{"name":"Department of Medical and Surgical Specialities, Radiological Sciences and Public Health, University of Brescia, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Maritati","sequence":"additional","affiliation":[{"name":"Nephrology, Dialysis and Transplant Unit, Ospedali Riuniti, Ancona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucio","family":"Manenti","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Parma University Hospital, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Palmisano","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Parma University Hospital, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giacomo","family":"Emmi","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Sciences, University of Firenze, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Romagnani","sequence":"additional","affiliation":[{"name":"Department of Biomedical Experimental and Clinical Sciences \u201cMario Serio\u201d, University of Firenze, and Nephrology Unit, Meyer Children\u2019s Hospital, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriella","family":"Moroni","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Policlinico Hospital, Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gina","family":"Gregorini","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Spedali Civili, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renato A.","family":"Sinico","sequence":"additional","affiliation":[{"name":"Nephrology Unit, University Milano Bicocca, Monza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R.W.","family":"Jayne","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Cambridge, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Augusto","family":"Vaglio","sequence":"additional","affiliation":[{"name":"Department of Biomedical Experimental and Clinical Sciences \u201cMario Serio\u201d, University of Firenze, and Nephrology Unit, Meyer Children\u2019s Hospital, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,9,6]]},"reference":[{"key":"2021020321154544500_sfaa139-B1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis 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