{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T00:17:48Z","timestamp":1792801068075,"version":"4.5.0"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T00:00:00Z","timestamp":1704153600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T00:00:00Z","timestamp":1704153600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"National Key Research and Development Program of China","award":["2018YFE0207300"],"award-info":[{"award-number":["2018YFE0207300"]}]},{"name":"CAMS Innovation Fund for Medical Sciences","award":["2021-I2M-1-005"],"award-info":[{"award-number":["2021-I2M-1-005"]}]},{"name":"Non-profit Central Research Institute Fund of Chinese Academy of Medical Science","award":["2019XK320022"],"award-info":[{"award-number":["2019XK320022"]}],"id":[{"id":"https:\/\/ror.org\/02drdmm93","id-type":"ROR","asserted-by":"crossref"}]},{"name":"National Natural Science Fund","award":["81771764, 81501414"],"award-info":[{"award-number":["81771764, 81501414"]}]},{"name":"Capital Health development Research Special Fund","award":["2016-4-40111"],"award-info":[{"award-number":["2016-4-40111"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hypertrophic cranial pachymeningitis (HCP) is uncommon but a poorly understood complication of granulomatosis with polyangiitis (GPA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>We conducted this retrospective study to elucidate the clinical characteristics and factors independently associated with granulomatosis with polyangiitis (GPA) complicated by hypertrophic cranial pachymeningitis (HCP) in China.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We collected the medical records of 78 patients diagnosed with GPA who were admitted to the inpatient department of Peking Union Medical College Hospital between January 2003 and September 2021. Clinical features, laboratory and radiological findings, and Birmingham Vasculitis Activity Scores (excluding meningitis score) were recorded. A binary logistic regression analysis was performed to analyze factors independently associated with GPA-related HCP.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Headache (100%) and cranial nerve palsy (61.5%) were common manifestations of HCP. Compared to 52 GPA patients without HCP, 26 patients with HCP required more time from initial symptoms to diagnosis, with a lower ratio of pulmonary and renal involvement, a higher ratio of myeloperoxidase\u2013antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, conductive or sensorineural hearing loss, mastoiditis, and decreased vision or sudden visual loss. Binary logistic regression analysis indicated that proteinase 3\u2013antineutrophil cytoplasmic antibody (PR3-ANCA) negativity (OR 10.698,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.001), conductive or sensorineural hearing loss (OR 10.855,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.005), and decreased vision or sudden visual loss (OR 8.647,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.015) were significantly associated with GPA-related HCP. Of the 26 patients, 18 received methylprednisolone pulse treatment, and 18 received intrathecal injections of dexamethasone and methotrexate.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>HCP was a severe manifestation of GPA in our study. Independent factors associated with the occurrence of HCP in patients with GPA included PR3-ANCA negativity, conductive or sensorineural hearing loss, and decreased vision or sudden visual loss. Furthermore, GPA-related HCP was associated with higher disease activity, requiring more intensive treatments.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13075-023-03239-9","type":"journal-article","created":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T01:02:37Z","timestamp":1704157357000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China"],"prefix":"10.1186","volume":"26","author":[{"given":"Yiran","family":"Chen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lijuan","family":"Gou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Qian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qingjun","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenjie","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengtao","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaofeng","family":"Zeng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fengchun","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,1,2]]},"reference":[{"issue":"3","key":"3239_CR1","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1093\/rheumatology\/keu336","volume":"54","author":"G De Luna","year":"2015","unstructured":"De Luna G, Terrier B, Kaminsky P, Le Quellec A, Maurier F, Solans R, et al. Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes. Rheumatology (Oxford). 2015;54(3):424\u201332.","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"3239_CR2","doi-asserted-by":"publisher","first-page":"724","DOI":"10.1007\/s00415-017-8416-0","volume":"264","author":"HA Choi","year":"2017","unstructured":"Choi HA, Lee MJ, Chung CS. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis. J Neurol. 2017;264(4):724\u201332.","journal-title":"J Neurol"},{"issue":"3","key":"3239_CR3","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1080\/03009742.2018.1498916","volume":"48","author":"T Sakairi","year":"2019","unstructured":"Sakairi T, Sakurai N, Nakasatomi M, Ikeuchi H, Kaneko Y, Maeshima A, et al. Hypertrophic pachymeningitis associated with antineutrophil cytoplasmic antibody-associated vasculitis: a case series of 15 patients. Scand J Rheumatol. 2019;48(3):218\u201324.","journal-title":"Scand J Rheumatol"},{"issue":"8","key":"3239_CR4","doi-asserted-by":"publisher","first-page":"1101","DOI":"10.1002\/art.1780330807","volume":"33","author":"RY Leavitt","year":"1990","unstructured":"Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener\u2019s granulomatosis. Arthritis Rheum. 1990;33(8):1101\u20137.","journal-title":"Arthritis Rheum"},{"issue":"2","key":"3239_CR5","doi-asserted-by":"publisher","first-page":"222","DOI":"10.1136\/ard.2006.054593","volume":"66","author":"R Watts","year":"2007","unstructured":"Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66(2):222\u20137.","journal-title":"Ann Rheum Dis"},{"issue":"3","key":"3239_CR6","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1136\/annrheumdis-2021-221794","volume":"81","author":"PA-O Grayson","year":"2022","unstructured":"Grayson PA-O, Ponte CA-O, Suppiah R, Robson JA-O, Craven A, Judge AA-O, et al. 2022 American College of Rheumatology\/European Alliance of Associations for rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):309\u201314.","journal-title":"Ann Rheum Dis."},{"key":"3239_CR7","doi-asserted-by":"crossref","unstructured":"Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023.","DOI":"10.1136\/ard-2022-223764"},{"issue":"1","key":"3239_CR8","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1186\/s13075-022-02898-4","volume":"24","author":"Y Shimojima","year":"2022","unstructured":"Shimojima Y, Kishida D, Ichikawa T, Kida T, Yajima N, Omura S, Nakagomi D, Abe Y, Kadoya M, Takizawa N, et al. Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS). Arthritis Res Ther. 2022;24(1):204.","journal-title":"Arthritis Res Ther"},{"issue":"1","key":"3239_CR9","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1097\/BOR.0b013e32834115f9","volume":"23","author":"JU Holle","year":"2011","unstructured":"Holle JU, Gross WL. Neurological involvement in Wegener\u2019s granulomatosis. Curr Opin Rheumatol. 2011;23(1):7\u201311.","journal-title":"Curr Opin Rheumatol"},{"issue":"4","key":"3239_CR10","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1111\/1756-185X.13046","volume":"20","author":"Y Shimojima","year":"2017","unstructured":"Shimojima Y, Kishida D, Hineno A, Yazaki M, Sekijima Y, Ikeda S-I. Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: a retrospective study of anti-neutrophil cytoplasmic antibody-associated vasculitis. Int J Rheum Dis. 2017;20(4):489\u201396.","journal-title":"Int J Rheum Dis"},{"issue":"8","key":"3239_CR11","doi-asserted-by":"publisher","first-page":"1467","DOI":"10.1007\/s00296-019-04338-0","volume":"39","author":"Y Gu","year":"2019","unstructured":"Gu Y, Sun X, Peng M, Zhang T, Shi J, Mao J. Pituitary involvement in patients with granulomatosis with polyangiitis: case series and literature review. Rheumatol Int. 2019;39(8):1467\u201376.","journal-title":"Rheumatol Int"},{"issue":"9","key":"3239_CR12","first-page":"111","volume":"30","author":"C Masson","year":"2021","unstructured":"Masson C, Boukriche Y, Colombani JM. Inflammatory hypertrophic cranial pachymeningitis. Presse Med. 2021;30(9):111\u20136.","journal-title":"Presse Med"},{"issue":"4","key":"3239_CR13","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1111\/head.13277","volume":"58","author":"MA Arnaoutoglou","year":"2018","unstructured":"Arnaoutoglou MA, Xerras CG, Kalevrosoglou IK, Rafailidis VD, Notas KP, Tegos TI. Headache linked to intracranial hypertension and hypertrophic pachymeningitis as the initial and dominant presentation of granulomatosis with polyangiitis. Case report and review of the recent literature. Headache. 2018;58(4):589\u201395.","journal-title":"Headache."},{"issue":"5","key":"3239_CR14","doi-asserted-by":"publisher","first-page":"885","DOI":"10.1016\/j.anl.2021.01.023","volume":"48","author":"Y Hosokawa","year":"2021","unstructured":"Hosokawa Y, Okada M, Suemori K, Hamaguchi N, Miyoshi KI, Takagi T, et al. The association between ear involvement and clinical features and prognosis in ANCA-associated vasculitis. Auris Nasus Larynx. 2021;48(5):885\u20139.","journal-title":"Auris Nasus Larynx"},{"issue":"1","key":"3239_CR15","doi-asserted-by":"publisher","first-page":"87","DOI":"10.1080\/14397595.2016.1177926","volume":"27","author":"Y Harabuchi","year":"2017","unstructured":"Harabuchi Y, Kishibe K, Tateyama K, Morita Y, Yoshida N, Kunimoto Y, et al. Clinical features and treatment outcomes of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV): a retrospective analysis of 235 patients from a nationwide survey in Japan. Mod Rheumatol. 2017;27(1):87\u201394.","journal-title":"Mod Rheumatol"},{"issue":"2 Suppl 41","key":"3239_CR16","first-page":"S60","volume":"24","author":"G Di Comite","year":"2006","unstructured":"Di Comite G, Bozzolo EP, Praderio L, Tresoldi M, Sabbadini MG. Meningeal involvement in Wegener\u2019s granulomatosis is associated with localized disease. Clin Exp Rheumatol. 2006;24(2 Suppl 41):S60\u20134.","journal-title":"Clin Exp Rheumatol"},{"issue":"Pt 2","key":"3239_CR17","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1093\/brain\/awt314","volume":"137","author":"A Yokoseki","year":"2014","unstructured":"Yokoseki A, Saji E, Arakawa M, Kosaka T, Hokari M, Toyoshima Y, et al. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain. 2014;137(Pt 2):520\u201336.","journal-title":"Brain"},{"key":"3239_CR18","doi-asserted-by":"publisher","first-page":"2855","DOI":"10.3389\/fimmu.2019.02855","volume":"10","author":"ZS Wallace","year":"2019","unstructured":"Wallace ZS, Stone JH. Personalized medicine in ANCA-associated vasculitis ANCA specificity as the guide? Front Immunol. 2019;10:2855.","journal-title":"Front Immunol"},{"issue":"12","key":"3239_CR19","doi-asserted-by":"publisher","first-page":"2945","DOI":"10.1002\/art.39812","volume":"68","author":"EM Miloslavsky","year":"2016","unstructured":"Miloslavsky EM, Lu N, Unizony S, Choi HK, Merkel PA, Seo P, et al. Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive and ANCA-negative patients with granulomatosis with polyangiitis (Wegener\u2019s): distinct patient subsets. Arthritis Rheumatol. 2016;68(12):2945\u201352.","journal-title":"Arthritis Rheumatol"},{"issue":"8","key":"3239_CR20","doi-asserted-by":"publisher","first-page":"1366","DOI":"10.1002\/art.41773","volume":"73","author":"SA Chung","year":"2021","unstructured":"Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology\/Vasculitis Foundation Guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021;73(8):1366\u201383.","journal-title":"Arthritis Rheumatol"},{"key":"3239_CR21","doi-asserted-by":"publisher","first-page":"608942","DOI":"10.5402\/2011\/608942","volume":"2011","author":"SA Just","year":"2011","unstructured":"Just SA, Knudsen JB, Nielsen MK, Junker P. Wegener\u2019s granulomatosis presenting with pachymeningitis: clinical and imaging remission by rituximab. ISRN Rheumatol. 2011;2011:608942.","journal-title":"ISRN Rheumatol."}],"container-title":["Arthritis Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-023-03239-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13075-023-03239-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-023-03239-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T13:36:49Z","timestamp":1704202609000},"score":1,"resource":{"primary":{"URL":"https:\/\/arthritis-research.biomedcentral.com\/articles\/10.1186\/s13075-023-03239-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,2]]},"references-count":21,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3239"],"URL":"https:\/\/doi.org\/10.1186\/s13075-023-03239-9","relation":{},"ISSN":["1478-6362"],"issn-type":[{"value":"1478-6362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,2]]},"assertion":[{"value":"15 April 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 December 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 January 2024","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 study protocol was approved by the Ethics Committee of Peking Union Medical College Hospital. The trial was conducted by the protocol and the provisions outlined in the Declaration of Helsinki. Because a review of medical records was performed for clinical purposes, the requirement for written informed consent was waived.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All authors have agreed to publish.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"6"}}