{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T22:28:34Z","timestamp":1778797714280,"version":"3.51.4"},"reference-count":40,"publisher":"BMJ","issue":"4","license":[{"start":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T00:00:00Z","timestamp":1767139200000},"content-version":"unspecified","delay-in-days":91,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2025,12,20]]},"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To investigate the concordance between organ involvement at diagnosis and relapse in granulomatosis with polyangiitis and factors associated with new disease features at relapse.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data from a national database of newly diagnosed patients was analysed. Clinical features were recorded at diagnosis and relapse, grouped by organ system. ORs and HRs were used to assess associations between baseline features and first relapse. Factors independently associated with new organ involvement at relapse were identified using multivariable logistic regression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 795 patients (median follow-up 3.5 years), 394 (50%) relapsed; organ involvement at relapse was available for 376 patients. Relapses most often affected ear, nose and throat (ENT), lungs and kidneys. Organ involvement at diagnosis was associated with a higher likelihood of relapse in the same organ: eyes (OR 6.69), lungs (OR 3.35), kidneys (OR 3.58), nervous system (OR 2.90), and mucocutaneous (OR 4.53). Major manifestations associated with a higher likelihood of recurrence were scleritis, pachymeningitis, subglottic stenosis and worsening renal function. For 56% of patients, the first relapse affected only the initially involved organs. Of the 165 patients with new organ manifestations, these were rarely isolated (n=34) and usually occurred alongside involvement of at least one previously affected organ (n=131). In multivariable analysis, systemic, ENT and lung manifestations at diagnosis were associated with a lower risk of new organ disease at relapse.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Although new features can still emerge, organ involvement at diagnosis is associated with a higher likelihood of relapse in the same organ.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/rmdopen-2025-006434","type":"journal-article","created":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T11:55:15Z","timestamp":1767182115000},"page":"e006434","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Concordance between presenting features and relapse in granulomatosis with polyangiitis: implications for risk assessment and counselling"],"prefix":"10.1136","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5871-8806","authenticated-orcid":false,"given":"Michele","family":"Iudici","sequence":"first","affiliation":[{"name":"Division of rheumatology, Geneva University Hospitals and University of Geneva, Geneva, Switzerland"},{"name":"Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zubeyir","family":"Salis","sequence":"additional","affiliation":[{"name":"University of Montpellier, Montpellier, France"},{"name":"Inserm U1046, CNRS UMR 9214, PhyMedExp, Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pascal","family":"Cohen","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antoine","family":"N\u00e9el","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Nantes University Hospital, and Nantes Universit\u00e9, INSERM, Center for Research in Transplantation and Translational Immunology (CR2TI), UMR 1064, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Achille","family":"Aouba","sequence":"additional","affiliation":[{"name":"D\u00e9partement de m\u00e9decine interne, CHU Caen, Caen, France, Caen, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francois","family":"Lifermann","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Dax Hospital, Dax, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc","family":"Ruivard","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, CHU Clermont-Ferrand, Institut Pascal, F-63000, Clermont-Ferrand, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Auma\u00eetre","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, CHU Clermont-Ferrand, Institut Pascal, F-63000, Clermont-Ferrand, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7229-3808","authenticated-orcid":false,"given":"Bernard","family":"Bonnotte","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases, Dijon-Bourgogne University Hospital, University EPE Bourgogne Europe, Dijon, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6547-232X","authenticated-orcid":false,"given":"Maxime","family":"Samson","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases, Dijon-Bourgogne University Hospital, University EPE Bourgogne Europe, Dijon, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francois","family":"Maurier","sequence":"additional","affiliation":[{"name":"Internal Medicine, UNEOS Hospitals, Metz-Ventoux, Metz, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Le Gallou","sequence":"additional","affiliation":[{"name":"Internal Medicine and Clinical Immunology, University of Rennes 1, Rennes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical immunology, National Referral Centre for Rare Systemic Auto-immune and auto-inflammatory Diseases, Univ. Lille, Inserm, CHU Lille, U1286 \u2013 INFINITE, University of Lille, Lille, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandre","family":"Karras","sequence":"additional","affiliation":[{"name":"Nephrology, European Hospital Georges Pompidou, AP-HP, University Paris-Cit\u00e9, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-0955","authenticated-orcid":false,"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[{"name":"National Referral Center for Rare Pulmonary Diseases, Louis-Pradel Hospital, Claude-Bernard University Lyon 1, Lyon, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9315-1577","authenticated-orcid":false,"given":"Noemie","family":"Jourde-Chiche","sequence":"additional","affiliation":[{"name":"APHM, CHU de la Conception, Nephrology, Aix-Marseille Univ, C2VN, INSERM, INRAE, Marseille, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Fran\u00e7ois","family":"Viallard","sequence":"additional","affiliation":[{"name":"Internal Medicine, Haut-L\u00e9v\u00eaque Hospital, University of Bordeaux, Bordeaux, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amandine","family":"Perier","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Niort Hospital, Niort, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pascal","family":"Godmer","sequence":"additional","affiliation":[{"name":"Internal Medicine, Bretagne - Atlantique Hospital, Vannes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Qu\u00e9m\u00e9neur","sequence":"additional","affiliation":[{"name":"Nephrology and Internal Medicine, Valenciennes Hospital, Valenciennes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1142-0978","authenticated-orcid":false,"given":"Claire","family":"de Moreuil","sequence":"additional","affiliation":[{"name":"Internal Medicine, La Cavale Blanche University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lo\u00efc","family":"Guillevin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6612-7336","authenticated-orcid":false,"given":"Benjamin","family":"Terrier","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3573-9203","authenticated-orcid":false,"given":"Xavier","family":"Pu\u00e9chal","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,12,31]]},"reference":[{"key":"2025123105201128000_11.4.e006434.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(23)01736-1"},{"key":"2025123105201128000_11.4.e006434.2","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.semarthrit.2021.02.002","article-title":"Granulomatosis with polyangiitis: Study of 795 patients from the French Vasculitis Study Group registry","volume":"51","author":"Iudici","year":"2021","journal-title":"Semin Arthritis Rheum"},{"key":"2025123105201128000_11.4.e006434.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2023.10.008"},{"key":"2025123105201128000_11.4.e006434.4","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2022-223764"},{"key":"2025123105201128000_11.4.e006434.5","doi-asserted-by":"crossref","DOI":"10.1186\/s13023-020-01621-3","article-title":"French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)","volume":"15","author":"Terrier","year":"2020","journal-title":"Orphanet J Rare Dis"},{"key":"2025123105201128000_11.4.e006434.6","doi-asserted-by":"publisher","DOI":"10.7326\/M19-3827"},{"key":"2025123105201128000_11.4.e006434.7","doi-asserted-by":"publisher","DOI":"10.1038\/s41584-022-00819-y"},{"key":"2025123105201128000_11.4.e006434.8","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2022-002953","article-title":"Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis","volume":"9","author":"Samson","year":"2023","journal-title":"RMD Open"},{"key":"2025123105201128000_11.4.e006434.9","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keq244"},{"key":"2025123105201128000_11.4.e006434.10","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.121473"},{"key":"2025123105201128000_11.4.e006434.11","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2021-221795"},{"key":"2025123105201128000_11.4.e006434.12","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"2025123105201128000_11.4.e006434.13","first-page":"S63","article-title":"Infliximab or rituximab for refractory Wegener\u2019s granulomatosis: long-term follow up. A prospective randomised multicentre study on 17 patients","volume":"29","author":"de Menthon","year":"2011","journal-title":"Clin Exp Rheumatol"},{"key":"2025123105201128000_11.4.e006434.14","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1404231"},{"key":"2025123105201128000_11.4.e006434.15","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2010.1658"},{"key":"2025123105201128000_11.4.e006434.16","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0802311"},{"key":"2025123105201128000_11.4.e006434.17","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1002\/art.39011","article-title":"Treatment of systemic necrotizing vasculitides in patients aged sixty-five years or older: results of a multicenter, open-label, randomized controlled trial of corticosteroid and cyclophosphamide-based induction therapy","volume":"67","author":"Pagnoux","year":"2015","journal-title":"Arthritis Rheumatol"},{"key":"2025123105201128000_11.4.e006434.18","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1803537"},{"key":"2025123105201128000_11.4.e006434.19","first-page":"671","article-title":"Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis","volume":"87","author":"Luqmani","year":"1994","journal-title":"QJM"},{"key":"2025123105201128000_11.4.e006434.20","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2006.062711"},{"key":"2025123105201128000_11.4.e006434.21","doi-asserted-by":"crossref","first-page":"1408","DOI":"10.1093\/ndt\/gfaa066","article-title":"PR3-ANCAs predict relapses in ANCA-associated vasculitis patients after rituximab","volume":"36","author":"van Dam","year":"2021","journal-title":"Nephrol Dial Transplant"},{"key":"2025123105201128000_11.4.e006434.22","doi-asserted-by":"crossref","first-page":"47","DOI":"10.2215\/CJN.06460622","article-title":"Maintenance of Remission and Risk of Relapse in Myeloperoxidase-Positive ANCA-Associated Vasculitis with Kidney Involvement","volume":"18","author":"Casal Moura","year":"2023","journal-title":"Clin J Am Soc Nephrol"},{"key":"2025123105201128000_11.4.e006434.23","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1093\/rheumatology\/keae409","article-title":"Relapses and serious adverse events during rituximab maintenance therapy in ANCA-associated vasculitis: a multicentre retrospective study","volume":"64","author":"Gialouri","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2025123105201128000_11.4.e006434.24","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2013.11.003"},{"key":"2025123105201128000_11.4.e006434.25","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2022.844112","article-title":"Do Relapses Follow ANCA Rises? A Systematic Review and Meta-Analysis on the Value of Serial ANCA Level Evaluation","volume":"9","author":"Al-Soudi","year":"2022","journal-title":"Front Med (Lausanne)"},{"key":"2025123105201128000_11.4.e006434.26","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1108735"},{"key":"2025123105201128000_11.4.e006434.27","doi-asserted-by":"crossref","DOI":"10.1155\/2009\/584916","article-title":"Monocyte chemoattractant protein 1 is a prognostic marker in ANCA-associated small vessel vasculitis","volume":"2009","author":"Ohlsson","year":"2009","journal-title":"Mediators Inflamm"},{"key":"2025123105201128000_11.4.e006434.28","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2018-214263"},{"key":"2025123105201128000_11.4.e006434.29","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1093\/rheumatology\/kex001","article-title":"Chronic nasal Staphylococcus aureus carriage identifies a subset of newly diagnosed granulomatosis with polyangiitis patients with high relapse rate","volume":"56","author":"Salmela","year":"2017","journal-title":"Rheumatology (Oxford)"},{"key":"2025123105201128000_11.4.e006434.30","doi-asserted-by":"crossref","first-page":"1448","DOI":"10.1136\/annrheumdis-2018-213645","article-title":"Characterisation of the nasal microbiota in granulomatosis with polyangiitis","volume":"77","author":"Rhee","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2025123105201128000_11.4.e006434.31","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2022.991256","article-title":"Environmental factors influencing the risk of ANCA-associated vasculitis","volume":"13","author":"Zhao","year":"2022","journal-title":"Front Immunol"},{"key":"2025123105201128000_11.4.e006434.32","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1111\/imm.13843","article-title":"The role and therapeutic strategies for tissue-resident memory T cells, central memory T cells, and effector memory T cells in psoriasis","volume":"173","author":"Deng","year":"2024","journal-title":"Immunology"},{"key":"2025123105201128000_11.4.e006434.33","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2023.1111521","article-title":"Tissue-resident memory T cells in renal autoimmune diseases","volume":"14","author":"Ginsberg","year":"2023","journal-title":"Front Immunol"},{"key":"2025123105201128000_11.4.e006434.34","unstructured":"Krebs CF , Reimers D , Zhao Y , et al . Pathogen-induced tissue-resident memory T(H)17 (T(RM)17) cells amplify autoimmune kidney disease. Sci Immunol 2020;5."},{"key":"2025123105201128000_11.4.e006434.35","doi-asserted-by":"crossref","first-page":"106933","DOI":"10.1016\/j.imlet.2024.106933","article-title":"The role of neutrophils in ANCA-associated vasculitis","volume":"270","author":"Prendecki","year":"2024","journal-title":"Immunol Lett"},{"key":"2025123105201128000_11.4.e006434.36","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1093\/rheumatology\/keab413","article-title":"Danger-associated molecular pattern molecules and the receptor for advanced glycation end products enhance ANCA-induced responses","volume":"61","author":"Page","year":"2022","journal-title":"Rheumatology (Oxford)"},{"key":"2025123105201128000_11.4.e006434.37","doi-asserted-by":"publisher","DOI":"10.1023\/B:JOCI.0000010422.73892.b5"},{"key":"2025123105201128000_11.4.e006434.38","doi-asserted-by":"publisher","DOI":"10.1172\/JCI65292"},{"key":"2025123105201128000_11.4.e006434.39","doi-asserted-by":"publisher","DOI":"10.1002\/1529-0131(200102)44:2<463::AID-ANR65>3.0.CO;2-9"},{"key":"2025123105201128000_11.4.e006434.40","doi-asserted-by":"crossref","first-page":"2464","DOI":"10.1093\/rheumatology\/keab719","article-title":"Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry","volume":"61","author":"Iudici","year":"2022","journal-title":"Rheumatology (Oxford)"}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2025-006434","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T13:20:20Z","timestamp":1767187220000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2025-006434"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10]]},"references-count":40,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2025,12,31]]},"published-print":{"date-parts":[[2025,10]]}},"alternative-id":["10.1136\/rmdopen-2025-006434"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2025-006434","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10]]}}}