{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T19:48:58Z","timestamp":1778096938854,"version":"3.51.4"},"reference-count":76,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","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":["K08-AR074562"],"award-info":[{"award-number":["K08-AR074562"]}],"id":[{"id":"10.13039\/100000069","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":["P30-AR070253"],"award-info":[{"award-number":["P30-AR070253"]}],"id":[{"id":"10.13039\/100000069","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":["R01-AR065538"],"award-info":[{"award-number":["R01-AR065538"]}],"id":[{"id":"10.13039\/100000069","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":["R01-AR073201"],"award-info":[{"award-number":["R01-AR073201"]}],"id":[{"id":"10.13039\/100000069","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":["R01-AR075906"],"award-info":[{"award-number":["R01-AR075906"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006260","name":"Rheumatology Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006260","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100017704","name":"Fundaci\u00f3n Bechara","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017704","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinical Immunology"],"published-print":{"date-parts":[[2020,6]]},"DOI":"10.1016\/j.clim.2020.108411","type":"journal-article","created":{"date-parts":[[2020,4,7]],"date-time":"2020-04-07T17:35:01Z","timestamp":1586280901000},"page":"108411","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":33,"special_numbering":"C","title":["Polyarteritis nodosa and deficiency of adenosine deaminase 2 \u2013 Shared genealogy, generations apart"],"prefix":"10.1016","volume":"215","author":[{"given":"Zhengping","family":"Huang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tianwang","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter A.","family":"Nigrovic","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pui Y.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.clim.2020.108411_bb0005","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1053\/berh.2002.0260","article-title":"Systemic vasculitides","volume":"16","author":"Gonzalez-Gay","year":"2002","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0010","series-title":"UpToDate","article-title":"Clinical manifestations and diagnosis of polyarteritis nodosa in adults","author":"Merkel","year":"2019"},{"key":"10.1016\/j.clim.2020.108411_bb0015","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1038\/nrrheum.2017.68","article-title":"The changing face of polyarteritis nodosa and necrotizing vasculitis","volume":"13","author":"Ozen","year":"2017","journal-title":"Nat. Rev. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0020","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1155\/2009\/258745","article-title":"HLA-DR in Brazilian patients with polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA)","volume":"26","author":"de Lira Freire","year":"2009","journal-title":"Dis. Markers"},{"key":"10.1016\/j.clim.2020.108411_bb0025","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1002\/art.1780260118","article-title":"HLA antigen frequencies in systemic vasculitis: increase in HLA-DR2 in Wegener\u2019s granulomatosis","volume":"26","author":"Elkon","year":"1983","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.clim.2020.108411_bb0030","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":"10.1016\/j.clim.2020.108411_bb0035","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":"10.1016\/j.clim.2020.108411_bb0040","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1056\/NEJMoa1307361","article-title":"Early-onset stroke and vasculopathy associated with mutations in ADA2","volume":"370","author":"Zhou","year":"2014","journal-title":"New Engl. J. Med."},{"key":"10.1016\/j.clim.2020.108411_bb0045","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1056\/NEJMoa1307362","article-title":"Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy","volume":"370","author":"Elkan","year":"2014","journal-title":"New Engl. J. Med."},{"key":"10.1016\/j.clim.2020.108411_bb0050","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1007\/s10875-018-0525-8","article-title":"Deficiency of adenosine deaminase 2 (DADA2): updates on the phenotype, genetics, pathogenesis, and treatment","volume":"38","author":"Meyts","year":"2018","journal-title":"J. Clin. Immunol."},{"key":"10.1016\/j.clim.2020.108411_bb0055","doi-asserted-by":"crossref","first-page":"282","DOI":"10.3389\/fped.2018.00282","article-title":"Vasculopathy, immunodeficiency, and bone marrow failure: the intriguing syndrome caused by deficiency of adenosine deaminase 2","volume":"6","author":"Lee","year":"2018","journal-title":"Front. Pediatr."},{"key":"10.1016\/j.clim.2020.108411_bb0060","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1002\/art.1780370206","article-title":"Nomenclature of systemic vasculitides. Proposal of an international consensus conference","volume":"37","author":"Jennette","year":"1994","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.clim.2020.108411_bb0065","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."},{"issue":"Suppl. 111","key":"10.1016\/j.clim.2020.108411_bb0070","first-page":"135","article-title":"Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda","volume":"36","author":"Karadag","year":"2018","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0075","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1007\/s11926-002-0026-9","article-title":"Historical perspective of vasculitis: polyarteritis nodosa and microscopic polyangiitis","volume":"4","author":"Matteson","year":"2002","journal-title":"Curr. Rheumatol. Rep."},{"key":"10.1016\/j.clim.2020.108411_bb0080","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1136\/ard.2009.116657","article-title":"EULAR\/PRINTO\/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria","volume":"69","author":"Ozen","year":"2010","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.clim.2020.108411_bb0085","doi-asserted-by":"crossref","first-page":"936","DOI":"10.1136\/ard.2005.046300","article-title":"EULAR\/PReS endorsed consensus criteria for the classification of childhood vasculitides","volume":"65","author":"Ozen","year":"2006","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.clim.2020.108411_bb0090","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1136\/ard.2009.116624","article-title":"EULAR\/PRINTO\/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: overall methodology and clinical characterisation","volume":"69","author":"Ruperto","year":"2010","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.clim.2020.108411_bb0095","series-title":"Ueber einige der wichtigsten Krankheiten der Arterien, Wien: Hof-Staatsdruckerei","first-page":"1","volume":"4","author":"K","year":"1852"},{"key":"10.1016\/j.clim.2020.108411_bb0100","first-page":"484","article-title":"Uber eine nicht bisher beschriebene eigenthumliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskelahmung einhergeht","volume":"1","author":"Kussmaul","year":"1866","journal-title":"Deutsche Archiv Klinische Medizin"},{"key":"10.1016\/j.clim.2020.108411_bb0105","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1002\/path.1700120107","article-title":"Polyarteritis acuta nodosa and periarteritis nodosa","volume":"12","author":"Dickson","year":"1908","journal-title":"J Pathol Bact"},{"key":"10.1016\/j.clim.2020.108411_bb0110","first-page":"350","article-title":"Ueber polyarteritis acuta nodosa (sogenannte periarteritis nodosa) und ihre beziehungen zur polymyositis und polyneuritis acuta","volume":"34","author":"Ferrari","year":"1903","journal-title":"Beitr Pathol Anat Allg Pathol"},{"key":"10.1016\/j.clim.2020.108411_bb0115","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1007\/s00467-005-1911-z","article-title":"Problems in classifying vasculitis in children","volume":"20","author":"Ozen","year":"2005","journal-title":"Pediatric. Nephrol. (Berlin, Germany)"},{"key":"10.1016\/j.clim.2020.108411_bb0120","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1002\/art.1780220803","article-title":"Polyarteritis nodosa in childhood a clinical pathologic study","volume":"22","author":"Ettlinger","year":"1979","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.clim.2020.108411_bb0125","doi-asserted-by":"crossref","first-page":"102","DOI":"10.3109\/14397595.2016.1172999","article-title":"Imbalanced expression of dysfunctional regulatory T cells and T-helper cells relates to immunopathogenesis in polyarteritis nodosa","volume":"27","author":"Shimojima","year":"2017","journal-title":"Mod. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0130","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1042\/BJ20050683","article-title":"Human ADA2 belongs to a new family of growth factors with adenosine deaminase activity","volume":"391","author":"Zavialov","year":"2005","journal-title":"Biochem. J."},{"key":"10.1016\/j.clim.2020.108411_bb0135","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1136\/annrheumdis-2016-210802","article-title":"ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: a multicentre national study","volume":"76","author":"Caorsi","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.clim.2020.108411_bb0140","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1002\/art.40913","article-title":"Identification of novel adenosine deaminase 2 gene variants and varied clinical phenotype in pediatric vasculitis","volume":"71","author":"Gibson","year":"2019","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.clim.2020.108411_bb0145","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.3389\/fimmu.2018.01361","article-title":"Deficiency of adenosine deaminase 2 in adult siblings: many years of a misdiagnosed disease with severe consequences","volume":"9","author":"Springer","year":"2018","journal-title":"Front. Immunol."},{"key":"10.1016\/j.clim.2020.108411_bb0150","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.3899\/jrheum.150024","article-title":"A Case series of adenosine deaminase 2-deficient patients emphasizing treatment and genotype-phenotype correlations","volume":"42","author":"Batu","year":"2015","journal-title":"J. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0155","doi-asserted-by":"crossref","first-page":"902","DOI":"10.1093\/rheumatology\/kev439","article-title":"Phenotypic variability in patients with ADA2 deficiency due to identical homozygous R169Q mutations","volume":"55","author":"Van Montfrans","year":"2016","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.clim.2020.108411_bb0160","doi-asserted-by":"crossref","first-page":"2314","DOI":"10.1002\/art.39699","article-title":"Deficiency of adenosine deaminase type 2: a description of phenotype and genotype in fifteen cases","volume":"68","author":"Nanthapisal","year":"2016","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.clim.2020.108411_bb0165","series-title":"Deficiency of Adenosine Deaminase 2 Triggers Adenosine-Mediated NETosis and TNF Production in Patients with DADA2 Blood","author":"Carmona-Rivera","year":"2019"},{"key":"10.1016\/j.clim.2020.108411_bb0170","doi-asserted-by":"crossref","first-page":"523","DOI":"10.3899\/jrheum.180045","article-title":"Variable clinical phenotypes and relation of interferon signature with disease activity in ADA2 deficiency","volume":"46","author":"Insalaco","year":"2019","journal-title":"J. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0175","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1186\/s12969-017-0193-x","article-title":"Autoimmune phenotype with type I interferon signature in two brothers with ADA2 deficiency carrying a novel CECR1 mutation","volume":"15","author":"Skrabl-Baumgartner","year":"2017","journal-title":"Pediatric Rheumatol. Online J."},{"key":"10.1016\/j.clim.2020.108411_bb0180","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2015-000236","article-title":"ADA2 deficiency: case report of a new phenotype and novel mutation in two sisters","volume":"2","author":"Uettwiller","year":"2016","journal-title":"RMD Open"},{"key":"10.1016\/j.clim.2020.108411_bb0185","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":"10.1016\/j.clim.2020.108411_bb0190","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1136\/ard.2006.054593","article-title":"Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies","volume":"66","author":"Watts","year":"2007","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.clim.2020.108411_bb0195","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.1002\/art.23470","article-title":"A paradigm of diagnostic criteria for polyarteritis nodosa: analysis of a series of 949 patients with vasculitides","volume":"58","author":"Henegar","year":"2008","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.clim.2020.108411_bb0200","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1007\/s11926-017-0699-8","article-title":"Deficiency of adenosine deaminase 2 (DADA2), an inherited cause of Polyarteritis Nodosa and a mimic of other systemic rheumatologic disorders","volume":"19","author":"Hashem","year":"2017","journal-title":"Curr. Rheumatol. Rep."},{"issue":"4","key":"10.1016\/j.clim.2020.108411_bb0205","doi-asserted-by":"crossref","first-page":"1365.e8","DOI":"10.1016\/j.jaci.2018.05.038","article-title":"Disrupted N-linked glycosylation as a disease mechanism in deficiency of ADA2","volume":"142","author":"Lee","year":"2018","journal-title":"J. Allergy Clin. Immunol."},{"key":"10.1016\/j.clim.2020.108411_bb0210","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1111\/cge.13518","article-title":"ADA2 deficiency due to a novel structural variation in 22q11.1","volume":"95","author":"Grossi","year":"2019","journal-title":"Clin. Genet."},{"issue":"1","key":"10.1016\/j.clim.2020.108411_bb0215","doi-asserted-by":"crossref","first-page":"117","DOI":"10.3899\/jrheum.181384","article-title":"A monogenic disease with a variety of phenotypes: deficiency of adenosine deaminase 2","volume":"47","author":"Ozen","year":"2020","journal-title":"J. Rheumatol."},{"issue":"2","key":"10.1016\/j.clim.2020.108411_bb0220","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1136\/annrheumdis-2019-216030","article-title":"Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis","volume":"79","author":"Lee","year":"2020","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.clim.2020.108411_bb0225","first-page":"536","article-title":"Polyarteritis nodosa and familial Mediterranean fever: a report of 2 cases and review of the literature","volume":"16","author":"Glikson","year":"1989","journal-title":"J. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0230","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/j.jpeds.2007.04.062","article-title":"Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa","volume":"151","author":"Yalcinkaya","year":"2007","journal-title":"J. Pediatr."},{"key":"10.1016\/j.clim.2020.108411_bb1000","article-title":"Genotype and functional correlates of disease phenotype in deficiency of adenosine deaminase 2 (DADA2)","volume":"13","author":"Lee","year":"2020","journal-title":"J Allergy Clin Immunol."},{"key":"10.1016\/j.clim.2020.108411_bb0235","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."},{"issue":"1","key":"10.1016\/j.clim.2020.108411_bb0240","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1097\/BOR.0000000000000669","article-title":"A monogenic autoinflammatory disease with fatal vasculitis: deficiency of adenosine deaminase 2","volume":"32","author":"Sahin","year":"2020","journal-title":"Curr. Opin. Rheumatol."},{"issue":"Suppl. 117","key":"10.1016\/j.clim.2020.108411_bb0245","first-page":"52","article-title":"Polyarteritis nodosa: lessons from 25 years of experience","volume":"37","author":"Sonmez","year":"2019","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0250","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.rdc.2014.09.005","article-title":"Polyarteritis nodosa","volume":"41","author":"Forbess","year":"2015","journal-title":"Rheum. Dis. Clin. N. Am."},{"key":"10.1016\/j.clim.2020.108411_bb0255","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/j.jaut.2014.01.029","article-title":"Diagnosis and classification of polyarteritis nodosa","volume":"48","author":"Hern\u00e1ndez-Rodr\u00edguez","year":"2014","journal-title":"J. Autoimmun."},{"key":"10.1016\/j.clim.2020.108411_bb0260","doi-asserted-by":"crossref","first-page":"1582","DOI":"10.1056\/NEJMc1801927","article-title":"Treatment strategies for deficiency of adenosine deaminase 2","volume":"380","author":"Ombrello","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.clim.2020.108411_bb0265","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":"10.1016\/j.clim.2020.108411_bb0270","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1189\/jlb.1109764","article-title":"Human adenosine deaminase 2 induces differentiation of monocytes into macrophages and stimulates proliferation of T helper cells and macrophages","volume":"88","author":"Zavialov","year":"2010","journal-title":"J. Leukoc. Biol."},{"key":"10.1016\/j.clim.2020.108411_bb0275","doi-asserted-by":"crossref","first-page":"4403","DOI":"10.1073\/pnas.062059699","article-title":"Adenosine deaminase-related growth factors stimulate cell proliferation in Drosophila by depleting extracellular adenosine","volume":"99","author":"Zurovec","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.clim.2020.108411_bb0280","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1136\/bmj.2.5054.1148","article-title":"The natural history of polyarteritis","volume":"2","author":"Rose","year":"1957","journal-title":"Br. Med. J."},{"key":"10.1016\/j.clim.2020.108411_bb0285","first-page":"43","article-title":"Polyarteritis nodosa","volume":"26","author":"Rose","year":"1957","journal-title":"QJM"},{"key":"10.1016\/j.clim.2020.108411_bb0290","series-title":"UpToDate","article-title":"Treatment and prognosis of polyarteritis nodosa","author":"Merkel","year":"2019"},{"key":"10.1016\/j.clim.2020.108411_bb0295","first-page":"537","article-title":"The effect of cortisone on the lesions of periarteritis nodosa","volume":"27","author":"Baggenstoss","year":"1951","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.clim.2020.108411_bb0300","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":"10.1016\/j.clim.2020.108411_bb0305","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 Care Res."},{"key":"10.1016\/j.clim.2020.108411_bb0310","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1016\/0002-9343(78)90533-8","article-title":"Cyclophosphamide-induced remissions in advanced polyarteritis nodosa","volume":"64","author":"Fauci","year":"1978","journal-title":"Am. J. Med."},{"key":"10.1016\/j.clim.2020.108411_bb0315","first-page":"405","article-title":"Treatment of polyarteritis nodosa and Churg-Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years","author":"Guillevin","year":"1992","journal-title":"Ann. Med. Interne"},{"key":"10.1016\/j.clim.2020.108411_bb0320","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":"10.1016\/j.clim.2020.108411_bb0325","doi-asserted-by":"crossref","first-page":"P270","DOI":"10.1186\/1546-0096-6-S1-P270","article-title":"Polyarteritis Nodosa (PAN) in childhood: a report of two siblings with intractable disease controlled by mycophenolate mofetil (MMF)","volume":"6","author":"Falcini","year":"2008","journal-title":"Pediatr. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0330","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1111\/j.1432-2277.1994.tb01577.x","article-title":"Polyarteritis nodosa type vasculitis in a patient with familial Mediterranean fever treated with cyclosporin a","volume":"7","author":"Henckes","year":"1994","journal-title":"Transpl. Int."},{"key":"10.1016\/j.clim.2020.108411_bb0335","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1093\/qjmed\/90.6.401","article-title":"Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis","volume":"90","author":"Adu","year":"1997","journal-title":"QJM"},{"key":"10.1016\/j.clim.2020.108411_bb0340","doi-asserted-by":"crossref","first-page":"978","DOI":"10.1093\/rheumatology\/kep148","article-title":"Biologic therapy in primary systemic vasculitis of the young","volume":"48","author":"Eleftheriou","year":"2009","journal-title":"Rheumatology (Oxford)"},{"issue":"10","key":"10.1016\/j.clim.2020.108411_bb0345","doi-asserted-by":"crossref","first-page":"2825","DOI":"10.1007\/s10067-019-04474-9","article-title":"Infliximab for the treatment of refractory polyarteritis nodosa","volume":"38","author":"Ginsberg","year":"2019","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0350","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2017-000446","article-title":"Treatment of polyarteritis nodosa with tocilizumab: a new therapeutic approach?","volume":"3","author":"Saunier","year":"2017","journal-title":"RMD Open"},{"key":"10.1016\/j.clim.2020.108411_bb0355","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1007\/s10067-005-0015-2","article-title":"High-dose intravenous immunoglobulin infusion in polyarteritis nodosa","volume":"25","author":"Asano","year":"2006","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0360","first-page":"S28","article-title":"Intravenous immunoglobulins in polyarteritis nodosa restricted to the limbs: case reports and review of the literature","volume":"25","author":"Balbir-Gurman","year":"2007","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.clim.2020.108411_bb0365","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":"10.1016\/j.clim.2020.108411_bb0370","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1002\/art.20401","article-title":"Short-term corticosteroids then lamivudine and plasma exchanges to treat hepatitis B virus\u2013related polyarteritis nodosa","volume":"51","author":"Guillevin","year":"2004","journal-title":"Arthritis Care Res."},{"key":"10.1016\/j.clim.2020.108411_bb0375","doi-asserted-by":"crossref","first-page":"2682","DOI":"10.1182\/blood-2017-07-798660","article-title":"Hematopoietic stem cell transplantation rescues the hematological, immunological, and vascular phenotype in DADA2","volume":"130","author":"Hashem","year":"2017","journal-title":"Blood"}],"container-title":["Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521661619306114?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521661619306114?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T16:02:17Z","timestamp":1761494537000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1521661619306114"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6]]},"references-count":76,"alternative-id":["S1521661619306114"],"URL":"https:\/\/doi.org\/10.1016\/j.clim.2020.108411","relation":{},"ISSN":["1521-6616"],"issn-type":[{"value":"1521-6616","type":"print"}],"subject":[],"published":{"date-parts":[[2020,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Polyarteritis nodosa and deficiency of adenosine deaminase 2 \u2013 Shared genealogy, generations apart","name":"articletitle","label":"Article Title"},{"value":"Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.clim.2020.108411","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}],"article-number":"108411"}}