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We herein describe a 4-year-old Chinese boy who was admitted for recurrent pneumonia and persistent hematuria and exhibited multisystem involvement and anti-neutrophil cytoplasmic antibody (ANCA) positivity. He was initially diagnosed with ANCA-associated vasculitis. However, genetic testing revealed a c.1574A&gt;G PIK3CD mutation, resulting in a diagnosis of APDS1.<\/jats:p>","DOI":"10.1177\/03000605211013222","type":"journal-article","created":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T02:27:02Z","timestamp":1622082422000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":13,"title":["Activated phosphoinositide 3-kinase delta syndrome misdiagnosed as anti-neutrophil cytoplasmic antibody-associated vasculitis: a case report"],"prefix":"10.1177","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3217-7105","authenticated-orcid":false,"given":"Xiaojing","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Nephrology, The Children\u2019s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, 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