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Following the Preferred Reporting Items for Systematic Reviews and Meta\u2010Analyses extension for scoping reviews, we searched MEDLINE and EMBASE for all peer\u2010reviewed articles using keywords including\u201cIgG4\u201d and \u201cmarginal zone lymphoma\u201d from their inception to February 20, 2022. Twenty\u2010two articles, including six observational studies and 24 cases from 16 case reports and case series, were included. Only one study had a comparative group, and the other five were exploratory observational studies. IgG4\u2010positive MZLs commonly occurred in males (83.3%). It primarily involved ocular adnexa (41.7%) and skin (29.2%). Only 29.2% had concurrent IgG4\u2010RD, and no expiration was noted. While most cases were treated with excision, resection, or clinical observation, 21.7% received rituximab\u2010cyclophosphamide, doxorubicin, vincristine, and prednisone as a first\u2010line treatment. This systematic review summarizes the current understanding of the characteristics of IgG4\u2010positive MZLs. While there seems to be IgG4\u2010RD\u2010related and <jats:italic>de novo<\/jats:italic> IgG4\u2010positive MZLs, future research needs to clearly define MZL with polyclonal IgG4\u2010positive cells and IgG4\u2010producing lymphoma. Further studies are critical to clarifying long\u2010term prognosis and optimal surveillance planning.<\/jats:p>","DOI":"10.1111\/pin.13251","type":"journal-article","created":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T09:04:32Z","timestamp":1654765472000},"page":"361-370","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Clinical characteristics and outcomes of IgG4\u2010positive marginal zone lymphoma: Systematic scoping review"],"prefix":"10.1111","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0224-7501","authenticated-orcid":false,"given":"Yoshito","family":"Nishimura","sequence":"first","affiliation":[{"name":"Department of Medicine, John A. 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Burns School of Medicine University of Hawai'i  Honolulu Hawaii USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Midori Filiz","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of Pathology Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences  Okayama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asami","family":"Nishikori","sequence":"additional","affiliation":[{"name":"Division of Pathophysiology Okayama University Graduate School of Health Sciences  Okayama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5234-6861","authenticated-orcid":false,"given":"Yasuharu","family":"Sato","sequence":"additional","affiliation":[{"name":"Division of Pathophysiology Okayama University Graduate School of Health Sciences  Okayama 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