{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T03:02:54Z","timestamp":1785380574976,"version":"3.55.0"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2021,8,12]],"date-time":"2021-08-12T00:00:00Z","timestamp":1628726400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"MHLW FC Program","award":["JPMH 20FC1044"],"award-info":[{"award-number":["JPMH 20FC1044"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,4,18]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>This study aimed to clarify the epidemiological and clinical features and treatment of patients with polyarteritis nodosa (PAN) in Japan.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We used the database of the Ministry of Health, Labour and Welfare (MHLW) of Japan in 2013 and 2014. We analysed 121 patients who were antineutrophil cytoplasmic antibodies negative among the patients certified as PAN according to the MHLW diagnostic criteria.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>The analysis included 60 males and 61 females, with a mean age of 52.9\u2009\u00b1\u200921.0\u2009years. As a general manifestation, fever was observed in 53.7%. Regarding organ involvement, skin manifestations (82.6%), joint and muscle manifestations (75.2%), and neuropsychiatric manifestations (50.4%) were common. Male patients had a higher proportion of mononeuritis multiplex involving motor neuropathy than female patients. Elderly patients had a higher proportion of general and respiratory manifestations. Glucocorticoids were used for treatment in all patients, and 19.0% underwent methylprednisolone pulse. Concomitant immunosuppressants were used in 25.6%, one-third of whom received cyclophosphamide. Methylprednisolone pulse and cyclophosphamide were mostly used in patients with life-threatening organ involvement.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>PAN developed in middle-aged people and led to numerous clinical manifestations. The common manifestations varied with age, and treatment was determined based on the type of organ involvement and disease severity.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/mr\/roab019","type":"journal-article","created":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T14:09:03Z","timestamp":1639231743000},"page":"598-605","source":"Crossref","is-referenced-by-count":7,"title":["Clinical characteristics of patients with polyarteritis nodosa based on a nationwide database in Japan"],"prefix":"10.1093","volume":"32","author":[{"given":"Mai","family":"Kawazoe","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, School of Medicine, Faculty of Medicine, Toho University, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshihiro","family":"Nanki","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, School of Medicine, Faculty of Medicine, Toho University, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noboru","family":"Hagino","sequence":"additional","affiliation":[{"name":"Division of Hematology and Rheumatology, Teikyo University Chiba Medical Center, Chiba, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriko","family":"Ikegaya","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Niigata Rheumatic Center, Niigata, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanari","family":"Kodera","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Rheumatology, Japan Community Health Care Organization Chukyo Hospital, Aichi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoko","family":"Nakano","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ehime Prefectural Central Hospital, Ehime, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miki","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Neurology, Tokyo Women\u2019s Medical University, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinya","family":"Kaname","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masayoshi","family":"Harigai","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Tokyo Women\u2019s Medical University School of Medicine, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,8,12]]},"reference":[{"key":"2022042010020955000_R1","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 Rheum"},{"key":"2022042010020955000_R2","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1253\/circj.CJ-19-0773","article-title":"JCS 2017 guideline on management of vasculitis syndrome - digest version","volume":"84","author":"Isobe","year":"2020","journal-title":"Circ J"},{"key":"2022042010020955000_R3","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1097\/MD.0b013e318205a4c6","article-title":"The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort","volume":"90","author":"Guillevin","year":"2011","journal-title":"Medicine (Baltimore)"},{"key":"2022042010020955000_R4","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1002\/art.20077","article-title":"Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener\u2019s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate","volume":"51","author":"Mahr","year":"2004","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R5","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1136\/ard.60.12.1156a","article-title":"Epidemiology of vasculitis in Europe","volume":"60","author":"Watts","year":"2001","journal-title":"Ann Rheum Dis"},{"key":"2022042010020955000_R6","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1002\/art.1780330805","article-title":"The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa","volume":"33","author":"Lightfoot","year":"1990","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R7","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1002\/art.27240","article-title":"Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database","volume":"62","author":"Pagnoux","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R8","doi-asserted-by":"crossref","first-page":"591","DOI":"10.3346\/jkms.2006.21.4.591","article-title":"Clinical features of polyarteritis nodosa in Korea","volume":"21","author":"Bae","year":"2006","journal-title":"J Korean Med Sci"},{"key":"2022042010020955000_R9","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1111\/1756-185X.12954","article-title":"Polyarteritis nodosa in north India: clinical manifestations and outcomes","volume":"20","author":"Sharma","year":"2017","journal-title":"Int J Rheum Dis"},{"key":"2022042010020955000_R10","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/s002960050016","article-title":"Outcome of polyarteritis nodosa in northern India","volume":"17","author":"Gupta","year":"1997","journal-title":"Rheumatol Int"},{"key":"2022042010020955000_R11","doi-asserted-by":"crossref","first-page":"884","DOI":"10.1002\/acr.23007","article-title":"Clinical spectrum of medium-sized vessel vasculitis","volume":"69","author":"Alibaz-Oner","year":"2017","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"2022042010020955000_R12","doi-asserted-by":"crossref","first-page":"1833","DOI":"10.1007\/s00296-018-4122-1","article-title":"A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts","volume":"38","author":"Karadag","year":"2018","journal-title":"Rheumatol Int"},{"key":"2022042010020955000_R13","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.5114\/aoms.2017.68407","article-title":"Polyarteritis nodosa: decreasing incidence in Poland","volume":"15","author":"Kanecki","year":"2019","journal-title":"Arch Med Sci"},{"key":"2022042010020955000_R14","first-page":"362","article-title":"Epidemiology of vasculitides in Khorasan Province, Iran","volume":"40","author":"Jokar","year":"2015","journal-title":"Iran J Med Sci"},{"key":"2022042010020955000_R15","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1093\/rheumatology\/kem107","article-title":"Prevalence of Wegener\u2019s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden","volume":"46","author":"Mohammad","year":"2007","journal-title":"Rheumatology (Oxford)"},{"key":"2022042010020955000_R16","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1097\/01.md.0000180792.80212.5e","article-title":"Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients","volume":"84","author":"Guillevin","year":"2005","journal-title":"Medicine (Baltimore)"},{"key":"2022042010020955000_R17","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1002\/art.11387","article-title":"Microscopic polyangiitis and polyarteritis nodosa: how and when do they start?","volume":"49","author":"Agard","year":"2003","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R18","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1007\/s00403-008-0898-2","article-title":"Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria","volume":"301","author":"Nakamura","year":"2009","journal-title":"Arch Dermatol Res"},{"key":"2022042010020955000_R19","doi-asserted-by":"crossref","first-page":"2476","DOI":"10.1002\/art.38024","article-title":"Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years","volume":"65","author":"Eleftheriou","year":"2013","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R20","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1111\/1756-185X.13120","article-title":"Comparing polyarteritis nodosa in children and adults: a single center study","volume":"20","author":"Erden","year":"2017","journal-title":"Int J Rheum Dis"},{"key":"2022042010020955000_R21","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1002\/1529-0131(200103)44:3<666::AID-ANR116>3.0.CO;2-A","article-title":"Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients","volume":"44","author":"Gayraud","year":"2001","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R22","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1002\/art.27340","article-title":"Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: a prospective randomized study of one hundred twenty-four patients","volume":"62","author":"Ribi","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R23","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1016\/0002-9343(79)90634-X","article-title":"Immunosuppressive and corticosteroid therapy of polyarteritis nodosa","volume":"67","author":"Leib","year":"1979","journal-title":"Am J Med"},{"key":"2022042010020955000_R24","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1056\/NEJM197908023010503","article-title":"Cyclophosphamide therapy of severe systemic necrotizing vasculitis","volume":"301","author":"Fauci","year":"1979","journal-title":"N Engl J Med"},{"key":"2022042010020955000_R25","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1002\/art.10922","article-title":"Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients","volume":"49","author":"Guillevin","year":"2003","journal-title":"Arthritis Rheum"},{"key":"2022042010020955000_R26","first-page":"51","volume-title":"2020 Treatment Guide for Anti-phospholipid Antibody Syndrome, Eosinophilic Granulomatosis with Polyangiitis, Polyarteritis Nodosa, and Rheumatoid Vasculitis (in Japanese)","author":"Kaname","year":"2021","edition":"1st"},{"key":"2022042010020955000_R27","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1097\/00005792-199601000-00003","article-title":"Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients","volume":"75","author":"Guillevin","year":"1996","journal-title":"Medicine (Baltimore)"},{"key":"2022042010020955000_R28","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1080\/14397595.2017.1337265","article-title":"Association of five-factor score with the mortality in Japanese patients with polyarteritis nodosa","volume":"28","author":"Abe","year":"2018","journal-title":"Mod Rheumatol"},{"key":"2022042010020955000_R29","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1136\/annrheumdis-2017-211796","article-title":"Prophylactic effect of trimethoprim-sulfamethoxazole for pneumocystis pneumonia in patients with rheumatic diseases exposed to prolonged high-dose glucocorticoids","volume":"77","author":"Park","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2022042010020955000_R30","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1007\/s00223-017-0335-7","article-title":"Glucocorticoids, inflammation and bone","volume":"102","author":"G\u00fcler-Y\u00fcksel","year":"2018","journal-title":"Calcif Tissue Int"},{"key":"2022042010020955000_R31","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1007\/s00774-014-0586-6","article-title":"Guidelines on the management and treatment of glucocorticoid-induced osteoporosis of the Japanese Society for Bone and Mineral Research: 2014 update","volume":"32","author":"Suzuki","year":"2014","journal-title":"J Bone Miner Metab"}],"container-title":["Modern Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/mr\/advance-article-pdf\/doi\/10.1093\/mr\/roab019\/41746161\/roab019.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mr\/article-pdf\/32\/3\/598\/43400494\/roab019.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mr\/article-pdf\/32\/3\/598\/43400494\/roab019.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T10:02:36Z","timestamp":1650448956000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/mr\/article\/32\/3\/598\/6348541"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,12]]},"references-count":31,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2021,8,12]]},"published-print":{"date-parts":[[2022,4,18]]}},"URL":"https:\/\/doi.org\/10.1093\/mr\/roab019","relation":{},"ISSN":["1439-7595","1439-7609"],"issn-type":[{"value":"1439-7595","type":"print"},{"value":"1439-7609","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,5,1]]},"published":{"date-parts":[[2021,8,12]]}}}