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The condition was originally characterized by livedoid rash, systemic inflammation, variable hypogammaglobulinemia, and early\u2010onset stroke. The phenotypic spectrum has expanded to include patients with immunodeficiency syndromes and bone marrow dysfunction, which are not typical features of <jats:styled-content style=\"fixed-case\">PAN<\/jats:styled-content>. Exploration into the pathogenesis and treatment options of <jats:styled-content style=\"fixed-case\">DADA<\/jats:styled-content>2 has added to our understanding of this condition, but more studies are needed. The purpose of this article is to review the various clinical phenotypes of <jats:styled-content style=\"fixed-case\">DADA<\/jats:styled-content>2, and raise awareness among rheumatologists to consider <jats:styled-content style=\"fixed-case\">DADA<\/jats:styled-content>2 when evaluating patients presenting with <jats:styled-content style=\"fixed-case\">PAN<\/jats:styled-content>\u2010like disease.<\/jats:p>","DOI":"10.1111\/1756-185x.13283","type":"journal-article","created":{"date-parts":[[2018,4,6]],"date-time":"2018-04-06T21:57:39Z","timestamp":1523051859000},"page":"69-77","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Diagnosis and management of <scp>ADA<\/scp>2 deficient polyarteritis nodosa"],"prefix":"10.1111","volume":"22","author":[{"given":"Andrea","family":"Human","sequence":"first","affiliation":[{"name":"Division of Rheumatology Department of Pediatrics BC Children's Hospital Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6287-9549","authenticated-orcid":false,"given":"Christian","family":"Pagnoux","sequence":"additional","affiliation":[{"name":"Vasculitis Clinic Mount Sinai Hospital Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,4,6]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1307361"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1307362"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40375"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38024"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.13120"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39122"},{"key":"e_1_2_9_8_1","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1136\/ard.2009.116657","article-title":"EULAR\/PRINTO\/PRES criteria for Henoch\u2010Sch\u00f6nlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. 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