{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T20:46:20Z","timestamp":1792442780708,"version":"4.5.0"},"reference-count":21,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T00:00:00Z","timestamp":1708560000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100018956","name":"NIHR Cambridge Biomedical Research Centre","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100018956","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["U54\u2010AR\u2010057319"],"award-info":[{"award-number":["U54\u2010AR\u2010057319"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000097","name":"National Center for Research Resources","doi-asserted-by":"publisher","award":["U54\u2010RR\u2010019497"],"award-info":[{"award-number":["U54\u2010RR\u2010019497"]}],"id":[{"id":"10.13039\/100000097","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL\u2010PAN).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with PAN were recruited between 1990 and 2020 from observational cohorts of nine countries across Europe, Japan, and North America. Eligibility was retrospectively defined using the European Medicines Agency classification algorithm. Patients with PAN related to hepatitis B virus (n\u00a0=\u00a012) and two monogenic diseases mimicking PAN, deficiency of adenosine deaminase 2 enzyme (n\u00a0=\u00a016) or familial Mediterranean fever (n\u00a0=\u00a011), were excluded. Data regarding organ involvement, relapse, disease\u2010related damage, and survival were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Three hundred fifty\u2010eight patients (female:male ratio 174:184), including those with systemic PAN (sPAN, n\u00a0=\u00a0282) and cutaneous PAN (n\u00a0=\u00a076), were included. Twenty\u2010five were pediatric onset. Mean \u00b1 SD age at diagnosis was 44.3 \u00b1\u200918.1\u2009years. Constitutional symptoms (71.5%), cutaneous involvement (70.5%), musculoskeletal findings (69.1%), and neurologic features (48.0%) were common manifestations. Among patients with sPAN, gastrointestinal involvement and proteinuria over 400 mg\/day were reported in 52.2% and 11.2%, respectively. During a median (interquartile range) 59.6 (99.5) months of follow\u2010up, relapse occurred in 48.5% of patients. One, 5\u2010 and 10\u2010year survival rates for sPAN were 97.1%, 94.0%, and 89.0%, respectively. Predictors of death for sPAN included age \u226565\u2009years at diagnosis, serum creatinine at diagnosis &gt;140 \u03bcmol\/L, gastrointestinal manifestations, and central nervous system (CNS) involvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The spectrum of PAN remains a complex, multifaceted disease. Relapse is common. Age \u226565\u2009years and serum creatinine &gt;140 \u03bcmol\/L at diagnosis, as well as gastrointestinal and CNS involvement, are independent predictors of death in sPAN.<\/jats:p>\n                    <jats:p>\n                      <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                        <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/art42817-toc-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.42817","type":"journal-article","created":{"date-parts":[[2024,2,12]],"date-time":"2024-02-12T03:28:59Z","timestamp":1707708539000},"page":"1120-1129","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study"],"prefix":"10.1002","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3443-3117","authenticated-orcid":false,"given":"Omer","family":"Karadag","sequence":"first","affiliation":[{"name":"Hacettepe University  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3886-2813","authenticated-orcid":false,"given":"Ertugrul Cagri","family":"Bolek","sequence":"additional","affiliation":[{"name":"Hacettepe University  Ankara 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