{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T07:50:42Z","timestamp":1794037842839,"version":"4.8.0"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100005639","name":"Universidade De S\u00e3o Paulo","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100005639","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Immunol Res"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Antineutrophil cytoplasmic antibody (ANCA)-associated pachymeningitis is a rare inflammatory disorder of the dura mater that may occur in isolation or as part of systemic vasculitis. The clinical features and outcomes of proteinase 3 (PR3)- and myeloperoxidase (MPO)-ANCA-associated pachymeningitis remain poorly defined. We aimed to synthesize clinical presentation, investigations, and treatment outcomes to improve diagnosis and guide management. A systematic review was conducted in accordance with PRISMA guidelines. PubMed, Embase, and Scopus were searched to June 15, 2023. Eligible studies were case reports or series of patients with imaging-confirmed pachymeningitis and positive ANCA serology. Individual patient data were extracted, including demographics, clinical features, pathology, and outcomes. We identified 230 patients from 177 reports, including 108 MPO-ANCA\u2013positive, 71 PR3-ANCA\u2013positive, and 3 dual MPO\/PR3-positive cases; 46 ANCA-positive cases had unspecified antigen specificity, and 2 were ELISA-negative but ANCA IIF-positive. Median age was 60 years; 53% were male. Headache and cranial neuropathies\u2014particularly hearing loss, visual impairment, and diplopia\u2014were predominant. Systemic involvement was present in 89% of cases, primarily affecting the ear, nose, throat, lungs, orbits and kidneys. Imaging often showed tentorium\/falx, middle cranial fossa, and frontal convexities involvement. Inflammatory markers were raised in &gt;\u200990%, and cerebrospinal fluid pleocytosis in 50%. Typical pathological features such as granulomas, vasculitis, or necrosis were documented in 66% of biopsied patients. At a median 9-month follow-up, mortality rate was 3.9% and relapse rate 23.5%. Rituximab (25%) was associated with lower rates of refractory disease. PR3- and MPO-ANCA-associated pachymeningitis typically presents with headache and cranial neuropathies, usually with systemic involvement. Relapse and incomplete recovery remain common despite treatment, underscoring the need for early recognition, targeted immunotherapy, and long-term follow-up.<\/jats:p>","DOI":"10.1007\/s12026-026-09782-2","type":"journal-article","created":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T02:20:28Z","timestamp":1777602028000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Antineutrophil cytoplasmic antibody-associated pachymeningitis: a systematic review of clinical features, diagnosis and treatment outcomes"],"prefix":"10.1007","volume":"74","author":[{"given":"Graziella Aguiar Santos","family":"Faria","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula Baleeiro Rodrigues","family":"Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela Abrah\u00e3o","family":"Allioni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leandro Tavares","family":"Lucato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tarso","family":"Adoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luiz Henrique Martins","family":"Castro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guilherme Diogo","family":"Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,1]]},"reference":[{"issue":"7","key":"9782_CR1","doi-asserted-by":"publisher","first-page":"732","DOI":"10.1136\/jnnp-2013-306410","volume":"85","author":"T Yonekawa","year":"2014","unstructured":"Yonekawa T, Murai H, Utsuki S, et al. A nationwide survey of hypertrophic pachymeningitis in Japan. J Neurol Neurosurg Psychiatry. 2014;85(7):732\u20139.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"9782_CR2","doi-asserted-by":"publisher","unstructured":"Terrim S, Mahler JV, Filho FVM, et al. Clinical presentation, investigation findings, and outcomes of IgG4-related pachymeningitis: a systematic review. JAMA Neurol Published online November. 2024;18. https:\/\/doi.org\/10.1001\/jamaneurol.2024.3947.","DOI":"10.1001\/jamaneurol.2024.3947"},{"issue":"23","key":"9782_CR3","first-page":"S63","volume":"99","author":"P Chakales","year":"2022","unstructured":"Chakales P, Herman M, Chien LC, Hutto S. Pachymeningitis in biopsy-proven sarcoidosis: clinical course, radiographic findings, response to treatment, and long-term outcomes. Neurology. 2022;99(23):S63\u20134.","journal-title":"Neurology"},{"issue":"5","key":"9782_CR4","doi-asserted-by":"publisher","first-page":"e300","DOI":"10.1016\/S2665-9913(24)00025-0","volume":"6","author":"S Arnold","year":"2024","unstructured":"Arnold S, Kitching AR, Witko-Sarsat V, et al. Myeloperoxidase-specific antineutrophil cytoplasmic antibody-associated vasculitis. Lancet Rheumatol. 2024;6(5):e300\u201313.","journal-title":"Lancet Rheumatol"},{"key":"9782_CR5","doi-asserted-by":"crossref","unstructured":"Damoiseaux J. (2021). ANCA testing in clinical practice: from implementation to quality control and harmonization. Front Immunol. 2021;12:656796.","DOI":"10.3389\/fimmu.2021.656796"},{"issue":"11","key":"9782_CR6","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1038\/nrrheum.2017.140","volume":"13","author":"X Bossuyt","year":"2017","unstructured":"Bossuyt X, Tervaert C, Arimura JW. Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683\u201392.","journal-title":"Nat Rev Rheumatol"},{"issue":"5","key":"9782_CR7","doi-asserted-by":"publisher","first-page":"e314","DOI":"10.1016\/S2665-9913(24)00035-3","volume":"6","author":"SD Falde","year":"2024","unstructured":"Falde SD, Fussner LA, Tazelaar HD, et al. Proteinase 3-specific antineutrophil cytoplasmic antibody-associated vasculitis. Lancet Rheumatol. 2024;6(5):e314\u201327.","journal-title":"Lancet Rheumatol"},{"issue":"6553","key":"9782_CR8","doi-asserted-by":"publisher","first-page":"eabf7844","DOI":"10.1126\/science.abf7844","volume":"373","author":"A Cugurra","year":"2021","unstructured":"Cugurra A, Mamuladze T, Rustenhoven J, et al. Skull and vertebral bone marrow are myeloid cell reservoirs for the meninges and CNS parenchyma. Science. 2021;373(6553):eabf7844.","journal-title":"Science"},{"issue":"6","key":"9782_CR9","doi-asserted-by":"publisher","first-page":"103338","DOI":"10.1016\/j.autrev.2023.103338","volume":"22","author":"Y Shimojima","year":"2023","unstructured":"Shimojima Y, Sekijima Y. Hypertrophic pachymeningitis in ANCA-associated vasculitis: Clinical and immunopathological features and insights. Autoimmun Rev. 2023;22(6):103338.","journal-title":"Autoimmun Rev"},{"key":"9782_CR10","doi-asserted-by":"publisher","first-page":"n71","DOI":"10.1136\/bmj.n71","volume":"372","author":"MJ Page","year":"2021","unstructured":"Page MJ, McKenzie JE, Bossuyt PM, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ. 2021;372:n71.","journal-title":"BMJ"},{"key":"9782_CR11","doi-asserted-by":"publisher","first-page":"180","DOI":"10.1164\/rccm.200507-1144OC","volume":"173","author":"KA Keogh","year":"2006","unstructured":"Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener\u2019s granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med. 2006;173:180\u20137.","journal-title":"Am J Respir Crit Care Med"}],"container-title":["Immunologic Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12026-026-09782-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12026-026-09782-2","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12026-026-09782-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T02:20:34Z","timestamp":1777602034000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12026-026-09782-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,1]]},"references-count":11,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["9782"],"URL":"https:\/\/doi.org\/10.1007\/s12026-026-09782-2","relation":{},"ISSN":["1559-0755"],"issn-type":[{"value":"1559-0755","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,1]]},"assertion":[{"value":"23 February 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 April 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 May 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"47"}}