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This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4\u2010positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri\u2010aortitis, coronary arteritis, and pericarditis. Intra\u2010cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4\u2010related disease with a long\u2010time lag between initiation of symptoms to proper diagnosis with biopsy\u2010proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports.<\/jats:p>","DOI":"10.1111\/echo.14828","type":"journal-article","created":{"date-parts":[[2020,8,26]],"date-time":"2020-08-26T07:08:40Z","timestamp":1598425720000},"page":"1478-1484","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["A challenging case report of IgG4\u2010related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions"],"prefix":"10.1111","volume":"37","author":[{"given":"Shokoufeh","family":"Hajsadeghi","sequence":"first","affiliation":[{"name":"Research Center for Prevention of Cardiovascular Disease Institute of Endocrinology &amp; Metabolism Iran University of Medical Sciences Tehran Iran"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9519-8854","authenticated-orcid":false,"given":"Marziyeh","family":"Pakbaz","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Disease Hazrat\u2010e Rasool General Hospital Iran University of Medical Sciences Tehran Iran"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Morteza","family":"Hassanzadeh","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Iran University of Medical Sciences Tehran Iran"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alireza","family":"Sadeghipour","sequence":"additional","affiliation":[{"name":"Department of Pathology and Oncopathology Research Center Iran University of Medical Sciences Tehran Iran"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,8,25]]},"reference":[{"doi-asserted-by":"publisher","key":"e_1_2_5_2_1","DOI":"10.2183\/pjab.94.027"},{"doi-asserted-by":"publisher","key":"e_1_2_5_3_1","DOI":"10.1016\/j.autrev.2010.05.003"},{"doi-asserted-by":"publisher","key":"e_1_2_5_4_1","DOI":"10.1080\/14397595.2018.1546367"},{"doi-asserted-by":"publisher","key":"e_1_2_5_5_1","DOI":"10.1007\/s00535-003-1175-y"},{"doi-asserted-by":"publisher","key":"e_1_2_5_6_1","DOI":"10.1097\/BOR.0000000000000133"},{"key":"e_1_2_5_7_1","first-page":"539","article-title":"IgG4\u2010related disease","volume":"63","author":"Ebbo M","year":"2013","journal-title":"Rev Du Prat"},{"key":"e_1_2_5_8_1","first-page":"1","article-title":"Development of an IgG4\u2010RD responder index. 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